Hyperinsulinemic Hypoglycemia, Familial, 7

PRESS RELEASE – NO. 1 / 2022 Zealand Pharma Announces Multiple Presentations at the 82nd Annual American Diabetes Association Scientific Sessions, Including Initial Clinical Data on GLP-1/GLP-2 Dual Agonist Dapiglutide A total of five presentations with Zealand Pharma products have been accepted for presentation at the American Diabetes Association (ADA) Abstracts include oral presentation of data from a Phase 1 Multiple Ascending Dose Trial with dapiglutide Data highlight and reinforce the Company’s commitment to advancing research and development of new treatment options for the management of diabetes and obesity Copenhagen, DK and Boston, MA, U.S. May 31, 2022 – Zealand Pharma A/S (Nasdaq: ZEAL) (CVR-no. 20045078), a biotechnology company focused on the discovery and development of innovative peptide-based medicines, today announced that three oral presentations and two poster abstracts with Zealand Pharma products have been accepted for the upcoming 82nd Scientific Sessions of the American Diabetes Association (ADA), which is being held in New Orleans from June 3-7, 2022. Zealand Pharma has two oral presentations, including Phase 1 clinical data demonstrating significant weight loss with Zealand’s dapiglutide, a novel GLP1/GLP2 dual agonist and preclinical data on the antidiabetic potential of the company’s long-acting amylin analogue ZP8396. Zealand also has a poster presentation with preclinical data highlighting the weight loss effects of ZP8396. Two additional abstracts include study results from a Zealand supported, investigator-initiated trial (IIT) with dasiglucagon led by investigators from the Steno Diabetes Center. This oral presentation reports novel data on the use of low dose dasiglucagon for prevention and treatment of non-severe hypoglycemia in people with type 1 diabetes. Additionally, Boehringer Ingelheim will have a poster presentation with preclinical data on the dual glucagon and glucagon-like peptide-1 receptor (GCGR/GLP-1R) agonist BI 456906 co-invented with Zealand Pharma, for the treatment of obesity. ZEALAND PHARMA PRESENTATION DETAILS Oral Presentation Title: Dapiglutide, a Once-Weekly GLP-1R/GLP-2R Dual Agonist, Was Safe and Well Tolerated and Showed Dose-Dependent Body Weight Loss over Four Weeks in Healthy SubjectsSession Title: Incretin Based TherapiesPresenting Author: Mikkel Agersnap MD, Medical Director – Clinical Development Presentation Date and Time: Monday, June 6th 5:00 - 5:15 PM (CDT) Session Location: Hall E-2 (Level 1)Abstract Number: 335-OR Oral Presentation Title: Antidiabetic Potential of Novel, Long-Acting Amylin Analogue ZP8396 in ZDF RatsSession Title: Lipids, Lipoproteins, and Diabetes (With ADA Presidents' Select Abstract Presentation)Presenting Author: Jolanta Skarbaliene, PhD, Principal Scientist – Clinical Pharmacology Presentation Date and Time: Sunday, June 5th , 2022, 6:00 - 6:15 PM (CDT)Location: 356 (Level 3) Abstract Number: 213-OR Poster Title: ZP8396, a Novel Amylin Analogue, Induces Weight Loss in DIO Rats with a Formulation Space at Physiological pHAuthor: Jolanta Skarbaliene, PhD, Principal Scientist – In vivo Pharmacology Poster Viewing Reception Date and Time: Monday, June 6th , 2022 12:00 – 1:00 PM (CDT)Location: Hall D-E Abstract Number: 1406-P ZEALAND SUPPORTED ITT WITH STENO DIABETES CENTER PRESENTATION DETAILS Oral Presentation Title: Pen-Administered Low-Dose Dasiglucagon vs. Usual Care for Prevention and Treatment of Nonsevere Hypoglycemia in People with Type 1 Diabetes During Free-Living Conditions: A Phase 2, Randomized, Two-Period, Crossover Outpatient StudySession Title: Other Therapeutic AgentsPresenting Author: Christian Laugesen, MD – Steno Diabetes CenterPresentation Date and Time: Monday, June 6th , 2022, 8:00 – 10:00 AM (CDT)Session Location: Hall E-3 (Level 1)Abstract Number: 257-OR BOEHRINGER INGELHEIM PRESENTATION DETAILS ON BI 456906 (co-invented with Zealand Pharma) Poster Title: The Dual Glucagon and Glucagon-Like Peptide-1 Receptor (GCGR/GLP-1R) Agonist BI 456906 Reduces Bodyweight in Diet-Induced Obese Mice Based on Food Intake Reduction and an Increase in Energy ExpenditurePresenting Author: Robert Augustin, PhD – Boehringer Ingelheim Poster Viewing Reception Date and Time: Sunday, June 5th, 2022, 12:00 - 01:00 PM (CDT) Location:Hall D-E Abstract Number: 723-P About DapiglutideDapiglutide (pINN) is a long-acting GLP-1R/GLP-2R dual agonist. The Phase 1b multiple-ascending dose, safety and tolerability trial investigating dapiglutide in healthy volunteers was completed in November 2021 and dapiglutide was found to have an acceptable safety and tolerability profile. Results showed a plasma half-life allowing for once weekly dosing and effects on several biomarkers suggest clinically relevant exposures of dapiglutide were achieved. About ZP8396ZP8396 is a long-acting amylin analogue designed to improve solubility, minimize fibrillation and allow for co-formulation with other peptides, including GLP-1 analogues. Amylin analogues hold potential as both mono and combination therapies for obesity. In preclinical studies ZP8396 has demonstrated potent anti-obesity effects and a Phase 1 program was initiated in 2021. About Dasiglucagon Mini-Dose Zealand is developing a dasiglucagon mini-dose pen for potential treatment of exercise-induced hypoglycemia in people living with type 1 diabetes and for people who suffer from meal-induced hypoglycemia following gastric bypass surgery. Clinical studies conducted in hospital settings have shown the potential for using low doses of dasiglucagon to correct moderate hypoglycemia. Top-line results from a Phase 2a dose-finding trial in people with type 1 diabetes were presented at the American Diabetes Association congress in June 2021, and top-line results of a post bariatric hypoglycemia Phase 2a trial were reported in 2020. Zealand is currently conducting two out-patient Phase 2 trials, utilizing the dasiglucagon mini-dose pen in in people with type 1 diabetes and for people that suffer from meal-induced hypoglycemia following gastric bypass surgery (ClinicalTrials.gov Identifier: NCT04764968 and NCT04836273). About BI 456906 BI 456906 is being investigated for the treatment of obesity and associated metabolic diseases. The dual glucagon (GCGR)/GLP1 receptor agonist activates both the GLP-1 and glucagon receptors and may offer better efficacy than currently available single-hormone receptor treatments. The compound leverages the known effects of the natural gut hormone oxyntomodulin, which has been shown to decrease food intake and increase energy expenditure in humans as well as the established effects of GLP-1 receptor agonists on both glucose control and body weight. About Zealand Pharma A/SZealand Pharma A/S (Nasdaq: ZEAL) ("Zealand") is a biotechnology company focused on the discovery and development of peptide-based medicines. More than 10 drug candidates invented by Zealand have advanced into clinical development, of which two have reached the market and three candidates are in late-stage development. In addition, license collaborations with Boehringer Ingelheim and AstraZeneca create opportunities for more patients to potentially benefit from Zealand-invented peptide investigational agents currently in development.Zealand was founded in 1998 in Copenhagen, Denmark and for more information about Zealand’s business and activities, please visit http://www.zealandpharma.com. About Boehringer Ingelheim Boehringer Ingelheim is working on breakthrough therapies that improve the lives of humans and animals. As a leading research-driven biopharmaceutical company, the company creates value through innovation in areas of high unmet medical need. Founded in 1885 and family-owned ever since, Boehringer Ingelheim takes a long-term perspective. Around 52,000 employees serve more than 130 markets in the three business areas, Human Pharma, Animal Health, and Biopharmaceutical Contract Manufacturing. Learn more at www.boehringer-ingelheim.com. Forward-Looking Statements This press release contains “forward-looking statements”, as that terms is defined in the Private Securities Litigation Reform Act of 1995, as amended, that provide Zealand Pharma’s expectations or forecasts of future events regarding the research, development and commercialization of pharmaceutical products. These forward-looking statements may be identified by words such as “aim,” “anticipate,” “believe,” “could,” “estimate,” “expect,” “forecast,” “goal,” “intend,” “may,” “plan,” “possible,” “potential,” “will,” “would” and other words and terms of similar meaning. You should not place undue reliance on these statements, or the scientific data presented. The reader is cautioned not to rely on these forward-looking statements. Such forward-looking statements are subject to risks, uncertainties and inaccurate assumptions, which may cause actual results to differ materially from expectations set forth herein and may cause any or all of such forward-looking statements to be incorrect, and which include, but are not limited to, the occurrence of adverse safety events; risks of unexpected costs or delays; unexpected concerns that may arise from additional data, analysis or results obtained during clinical trials; failure to protect and enforce our data, intellectual property and other proprietary rights and uncertainties relating to intellectual property claims and challenges; regulatory authorities may require additional information or further studies, or may fail to approve or may delay approval of our drug candidates or expansion of product labelling; failure to obtain regulatory approvals in other jurisdictions; product liability claims; and the direct and indirect impacts of the ongoing COVID-19 pandemic on our business, results of operations and financial condition. If any or all of such forward-looking statements prove to be incorrect, our actual results could differ materially and adversely from those anticipated or implied by such statements. The foregoing sets forth many, but not all, of the factors that could cause actual results to differ from our expectations in any forward-looking statement. All such forward-looking statements speak only as of the date of this press release and are based on information available to Zealand Pharma as of the date of this release. We do not undertake to update any of these forward-looking statements to reflect events or circumstances that occur after the date hereof. Information concerning pharmaceuticals (including compounds under development) contained within this material is not intended as advertising or medical advice. For further information, please contact: Zealand Pharma Investor RelationsMaeve ConneightonArgot Partnersinvestors@zealandpharma.com Zealand Pharma Media Relations David Rosen Argot Partnersmedia@zealandpharma.com

Company announcement – No. 67/ 2021 Interim report for 9M 2021 Zealand Pharma Presents Financial Results for the First Nine Months of 2021; Provides Updates on Commercial Launch and Timing for Congenital Hyperinsulinism and Short Bowel Syndrome Phase Three Clinical Studies Copenhagen, DK and Boston, MA, U.S. November 11, 2021 – Zealand Pharma A/S (Nasdaq: ZEAL) (CVR-no. 20045078) a biotechnology company focused on the discovery, development, and commercialization of innovative peptide-based medicines, today announced financial results for the first nine months of 2021. Financial results for the first nine months of 2021 Revenue: DKK 238.6 million / USD 37.1 million (DKK 290.0 million / USD 45.6 million in the first nine months of 2020). Net operating expenses: DKK -906.2 million / USD -141.1 million (DKK -714.5 million / USD -112.3 million in the first nine months of 2020). Net operating result: DKK -762.6 million / USD -118.8 million (DKK -449.1 million / USD -70.6 million in the first nine months of 2020). Cash, cash equivalents, and marketable securities: DKK 1,049.0 million / USD 163.3 million as of September 30, 2021 (September 30, 2020: DKK 1,528.6 million / USD 240.4 million). Business highlights for the first nine months of 2021 Announced collaboration agreement with DEKA Research & Development Corp. to advance development of infusion pump to be used with dasiglucagon for the potential treatment of congenital hyperinsulinism (CHI).Zealand Pharma A/S entered into a definitive collaboration agreement with DEKA Research & Development Corp., to develop a continuous infusion pump to be used in combination with dasiglucagon, an investigational agent currently in phase 3 trials in patients with congenital hyperinsulinism. The agreement covers the technical development of the pump system as well as associated commercialization activities. Announced presentation of preclinical data on Amylin Analogue ZP8396 and clinical data on GLP1-Glucagon Dual-Agonist BI 456906 at The Obesity Society Annual Meeting. Key findings from the preclinical study of amylin analogue ZP8396 were: monotherapy showed comparable weight loss potential, with the combination therapy being superior for the treatment of obesity and obesity related comorbidities; and administration of amylin analogue ZP8396 in combination with semaglutide, a GLP-1 analogue, resulted in increased food intake inhibition and body weight loss (~20% vs initial body weight) as compared to single treatment alone in the diet induced obese (DIO) in vivo models. Key findings of the Phase 1 trial with BI 456906, a subcutaneous dual GLP-1R/GCGR agonist were in the Phase I dose escalation study; BI 456906 was generally well tolerated and resulted in clinically relevant bodyweight reductions of up to 6.6% after 6 weeks and 13.7% after 16 weeks. Announced first patient dosed in EASE-SBS 4 phase 3b trial assessing Glepaglutide in patients with Short Bowel Syndrome. The EASE-SBS 4 Phase 3b trial is part of Zealand’s EASE-SBS Phase 3 program for glepaglutide. (ClinicalTrials.gov identifier (NCT number): NCT04991311) and will evaluate long-term effects on intestinal absorption of fluids and energy of once weekly glepaglutide injections. EASE-SBS 4 is an open-label single-center Phase 3b trial investigating the long-term effect on intestinal absorption, nutritional status and long-term safety of administration of glepaglutide in patients with short bowel syndrome (SBS). Ten patients will receive once weekly 10 mg subcutaneous injections of glepaglutide over 26 weeks. The EASE-SBS 1 pivotal Phase 3 trial remains on track for trial results to be obtained in 2022. Emmanuel Dulac, President and Chief Executive Officer at Zealand Pharma, comments: “Zealand Pharma has continued to undergo a transformational first nine months in 2021 and developed into an integrated biopharmaceutical company by leveraging our innovative peptide platform to address unmet needs in type 1 diabetes management and rare diseases. We are progressing with the early stages of our commercial launch of ZEGALOGUE® (dasiglugacon) injection with Zegalogue currently available to 65% of all commercial lives. As our commercial team ramps up the ZEGALOGUE commercial launch, we also look forward to building upon the momentum to advance our other pipeline programs. Recently, we have been encouraged by preclinical data from ZP8396, our Amylin Analogue, and clinical data from GLP-1, our Glucagon Dual-Agonist BI 456906, both sets presented at the recent The Obesity Society Annual Meeting. Additionally, we entered into a collaboration agreement with DEKA Research & Development Corp. and are working to advance the development of their infusion pump to be used with dasiglucagon as a potential treatment option of CHI. Finally, while the Zegalogue net revenue projections have not materialized as we had hoped and we have lowered our revenue guidance as a result, we remain confident in the work that we have done to set the stage for Zegalogue to have commercial success in 2022 and beyond.” Financial guidance for 2021 Net product revenue from the sales of commercial products is expected to be DKK 190 million +/- 10%. This is a decrease of DKK 30 million from the guidance issued on March 11, 2021. The reduction in net revenue from the previous guidance is driven by lower-than-expected sales of Zegalogue for 2021. In 2021, Zealand Pharma expects revenue from existing license agreements. However, since such revenue is uncertain in terms of size and timing, Zealand Pharma does not intend to provide guidance on such revenue. Net operating expenses in 2021 are expected to be DKK 1,250 million +/-10% and remains unchanged from the financial guidance issued on March 11, 2021. Update regarding COVID-19 Zealand Pharma continues to monitor the COVID-19 pandemic and take precautions to keep our employees, patients, business and clinical partners safe. This is an ongoing exercise in monitoring the effects of the pandemic on all of our key stakeholders and responding appropriately. We maintain compliance with guidance from applicable government and health authorities as appropriate. We have adapted the way we work to support our community’s efforts to reduce the transmission of COVID-19 and protect our employees, while continuing to provide patient care and maintain business continuity. Zealand Pharma has taken measures to secure its discovery activities, which remain ongoing, while work in laboratories and offices has been organized to reduce the risk of COVID-19 transmission. The impact of COVID-19 on our research activities has thus far been minimal. Employees who can work from home have been doing so, while those needing to work in laboratory facilities are divided into shifts to reduce the number of people gathered at one time. Business travel has been minimized and online and video conference technology is used to meet virtually rather than in person. We have continued our clinical trials while working with authorities, investigators, trial sites and contract research organizations to minimize site visits and ensure optimal trial follow-up. In late April, Zealand Pharma in Denmark commenced a gradual return to office program, which currently allows up to 60% occupancy, consistent with local health authority guidelines and practice including frequent testing, sanitizing, and other protective measures. COVID-19 restrictions have not affected our phase 3-program for dasiglucagon in congenital hyperinsulinism (CHI) and we expect topline data from the second Phase 3 trial in 2021. The pandemic impacted the speed of patient recruitment for our Phase 3 trial with glepaglutide for the treatment of short bowel syndrome, and results are expected in 2022. We currently do not anticipate changes to the timelines for the bi-hormonal artificial pancreas pump Phase 3 program. Our research and in particular our development programs may be impacted if the pandemic continues to put increased pressure on hospital systems, slow recruitment of patients into the trials or cause lockdowns that affect our clinical trial sites if key external medical resources are diverted elsewhere. Direct engagement with health care providers and patients has been reduced and transformed by leveraging virtual meetings, training, and support. Our commercial team is focused on supporting the businesses for the V-Go® wearable insulin delivery device and Zegalogue while ensuring a continued high level of service and support for existing patients. Commercial Update Zegalogue® (dasiglucagon) injection Zegalogue (dasiglucagon) injection was approved by the U.S. FDA on March 22, 2021 for the treatment of severe hypoglycemia in people with diabetes aged 6 and over. Zegalogue is available in both an auto injector and a prefilled syringe. The approval was based on efficacy results from three pivotal trials in adults and children with type 1 diabetes, whereby the primary endpoint of time to plasma glucose recovery, was successfully achieved with a median time to blood glucose recovery of 10 minutes following Zegalogue administration. In these Phase 3 studies, the most common adverse events reported (≥2%) were nausea, vomiting, headache, diarrhea, and injection site pain in adults; and nausea, vomiting, headache and injection site pain in pediatric patients. Zegalogue launched in the U.S. in late June 2021. The Company’s primary goal during launch is to ensure all eligible patients have access to Zegalogue and feel supported in their treatment plan. Since approval, Zealand Pharma has made substantial progress working with Pharmacy Benefit Managers (PBMs), Managed Care Organizations, and state Medicaid agencies to add Zegalogue to their respective formularies. As a result of this work, Zegalogue now has unrestricted coverage in approximately 65% of commercial lives which accounts for more than 120 million lives and approximately 55% of Medicaid lives, which accounts for 40 million Medicaid lives. Beginning in 2022, we expect Zegalogue will have unrestricted coverage in approximately 70% of commercial and Medicaid lives, setting the stage for a sustained acceleration of growth. Zegalogue net revenue for the period of July 1 – September 30, 2021 was DKK 3.1 million / USD $0.5 million. Year-to-Date net revenue is DKK 4.3 million / USD $0.7 million. For the year, net product revenue of Zegalogue is anticipated to be below our initial revenue targets. The reduction in revenue is a result of lower-than-expected uptake of prescriptions due to the increase in timelines for approved drug coverage agreements with payers to translate into approved prescriptions in pharmacies. V-Go® wearable insulin delivery device The V-Go series of Wearable Insulin Delivery Devices are indicated for continuous subcutaneous infusion of either 20 Units of insulin (0.83 U/hr), 30 Units of insulin (1.25 U/hr) or 40 Units of insulin (1.67 U/hr) in one 24-hour time period and on-demand bolus dosing in 2-Unit increments (up to 36 Units per one 24-hour time period) in adults requiring insulin. V-Go net revenue for the period of July 1 – September 30, 2021 was DKK 49.1 million / USD $7.8 million. Year-to-Date net revenue is DKK 139.0 million / USD $22.4 million. Pipeline Update Type 1 Diabetes Management Dasiglucagon for bihormonal Artificial Pancreas systems Zealand Pharma is developing a pre-filled dasiglucagon cartridge intended for use in Bihormonal Artificial Pancreas systems, which holds potential to improve the management of type 1 diabetes (T1D). Zealand is collaborating with Beta Bionics, developer of the bihormonal iLet® bionic pancreas system, a pocket-sized, dual-chamber (insulin and glucagon), autonomous, glycemic control system. The iLet® bionic pancreas is an investigational device, limited by federal (or United States) law to investigational use only. The iLet® bionic pancreas intends to mimic a biological pancreas by calculating and dosing insulin and/or glucagon (dasiglucagon) as needed, based on input data from a continuous glucose monitor (CGM) worn by a person with diabetes. Top-line results from a Phase 2, pre-pivotal trial in people with type 1 diabetes showed that the bihormonal iLet® bionic pancreas using insulin and dasiglucagon provided improved glycemic control relative to the iLet® bionic pancreas delivering insulin alone. During the bihormonal and insulin-only periods, 90% (9 of 10) and 50% (5 of 10) of participants, respectively, had a mean CGM glucose level of < 154 mg/dL.1,2 Importantly these glucose levels were achieved while the median time with CGM glucose levels < 54 mg/dL was only 0.2% during the bihormonal period and 0.6% during the insulin-only period. Zealand’s partner, Beta Bionics, and the study sponsor, the Jaeb Center for Health Research, are on track to begin screening in the fourth quarter 2021 into the Phase 3, Bihormonal iLet® Bionic Pancreas Pivotal Program, utilizing insulin and dasiglucagon. The program includes three sub-trials, which are anticipated to provide the clinical data necessary to support the market application for the bihormonal iLet® bionic pancreas and the new-drug application (NDA) for the use of dasiglucagon in bihormonal Artificial Pancreas systems. The first of these three sub-trials will be a three-month single-arm, bihormonal-only safety and test-run trial that will enroll two participants at each of the approximately 30 clinical sites. After 20 pediatric participants and 20 adult participants have been successfully treated for a minimum of 3 weeks in this trial, the two randomized controlled trials (RCTs) will begin – one enrolling ~ 350 pediatric participants (6–17 years of age) and the other enrolling 350 adult participants (≥ 18 years of age) with T1D. The primary outcome measure in the RCTs is superiority in HbA1c of the bihormonal iLet® bionic pancreas using dasiglucagon relative to the insulin-only iLet® system after 26 weeks of therapy on the two interventions. The bihomonal iLet® bionic pancreas performance will also be compared to intensified usual care using CGM therapy in a third arm in both the pediatric and adult RCTs. Dasiglucagon mini-dose pen Zealand is developing a dasiglucagon mini-dose pen for potential treatment of exercise-induced hypoglycemia in people living with type 1 Diabetes and for people who suffer from meal-induced hypoglycemia following gastric bypass surgery. Clinical studies conducted in hospital settings have shown the potential for using low doses of dasiglucagon to correct moderate hypoglycemia. Top-line results from a Phase 2a dose-finding trial in people with type 1 diabetes were presented at the American Diabetes Association congress in June 2021, and top-line results of a post bariatric hypoglycemia Phase 2a trial were reported in 2020. Out-patient Phase 2 trials in exercise-induced hypoglycemia in people living with type 1 diabetes and for people that suffer from meal-induced hypoglycemia following gastric bypass surgery were initiated in the second quarter this year (ClinicalTrials.gov Identifier: NCT04764968 and NCT04836273). The Phase 2 trial in people with T1D specifically evaluates the effectiveness of pen-administered low-dose dasiglucagon for prevention and treatment of hypoglycemia in people with type 1 diabetes. Rare Diseases Dasiglucagon for congenital hyperinsulinism (CHI) The potential for chronic dasiglucagon infusion delivered via a pump to prevent hypoglycemia in children with CHI is being evaluated in a Phase 3 program. The aim is to reduce or eliminate the need for intensive hospital treatment, reduce the frequency of dangerous low blood glucose and need for constant feeding, and to potentially delay or eliminate the need for pancreatectomy. The FDA and the European Commission both granted orphan drug designation to dasiglucagon for the treatment of CHI. We announced data from the first Phase 3 trial in the program, trial 17109, in December 2020. This trial evaluated children aged 3 months to 12 years old with more than three hypoglycemic events per week despite previous near-total pancreatectomy and/or maximum medical therapy. Dasiglucagon on top of standard of care (SOC) did not significantly reduce the rate of hypoglycemia compared to SOC alone when assessed by the primary endpoint, intermittent self-measured plasma glucose. However, hypoglycemia was reduced by 40–50% with dasiglucagon as compared to SOC alone when assessed by blinded continuous glucose monitoring. Dasiglucagon treatment was assessed to be well tolerated in the study and 31 out of 32 patients continued into the long-term extension study. We are conducting additional analyses and engaging with regulatory authorities to discuss the results of 17109 while awaiting the outcome of a second Phase 3 trial, 17103, in neonates up to 12 months old with CHI. We expect to complete enrolment into the 17103 trial by the end of 2021 and have results in the first half of 2022. Glepaglutide for short bowel syndrome (SBS) Glepaglutide is a long-acting GLP-2 analog, being developed in an auto-injector with potential for convenient weekly administration for reducing or eliminating the need for parenteral support in people living with SBS. EASE-SBS 1 is the pivotal Phase 3 trial with enrolment of up to 129 patients with SBS that seeks to establish the efficacy and safety of once- and twice-weekly administration of glepaglutide. Patients will be treated for six months in EASE-SBS 1, whereafter they are offered four years continuous treatment with glepaglutide in the extension trials, EASE-SBS 2 and 3. A Phase 3b trial, EASE-SBS 4, was initiated in Q3 2021 and will assess long-term effects of glepaglutide on intestinal fluid and energy uptake. The primary endpoint in EASE-SBS 1 is the absolute reduction in parenteral support achieved by the end of the trial. Based on a dialogue with FDA and EMA we have decided to introduce an interim analysis that could allow for an early stopping of the study for efficacy or futility before the full 129 patients have been enrolled. If the interim readout meets the criteria for early stopping, we plan to pursue an NDA submission as a next development step based on these clinical data. We expect to have all subjects needed for the interim analysis enrolled by the end of 2021 with results of the interim analysis being available in Q3 2022. The U.S. FDA granted orphan drug designation to glepaglutide for the treatment of SBS. Dapiglutide Dapiglutide (pINN) is a long-acting GLP-1R/GLP-2R dual agonist. The Phase 1a single-ascending dose, safety and tolerability trial investigating dapaglutide in healthy volunteers was completed in Q3 2020 and dapiglutide was found to have an acceptable safety and tolerability profile. Results showed a plasma half-life allowing for once weekly dosing. Based on the results of the Phase 1a trial, Zealand initiated a Phase 1b (multiple ascending dose) safety and tolerability trial and all subjects have received the last dapiglutide dose for the safety and tolerability trial with key results expected in the fourth quarter of 2021 (ClinicalTrials.gov Identifier: NCT04612517). Obesity Amylin ZP8396 is a potent long-acting amylin analogue designed to improve solubility and allow for co-formulation with other peptides, including GLP1 analogues. Amylin analogues hold potential as both mono and combination therapies for obesity. We have initiated a Phase 1 clinical trial with ZP8396 for potential treatment of obesity. The Phase 1, First-in-Human, randomized, single ascending dose trial will assess the safety, tolerability, pharmacokinetics, and pharmacodynamics of ZP8396 administered to healthy subjects. Preclinical data on ZP8396 was presented at The Obesity Society Annual Meeting, which showed anti-obesity effects of ZP8396 in in vivo models, with up to 20% weight loss when combined with GLP1 analogue semaglutide. ZP8396 is also being developed as a potential treatment for type 2 diabetes. BI 456906: Long-acting GLP-1/GLU dual agonist for obesity and/or diabetes (with Boehringer Ingelheim) The GLP-1/glucagon dual agonist activates two key gut hormone receptors simultaneously and may offer better blood sugar and weight-loss control than current single-hormone receptor agonist treatments. The lead molecule BI 456906 is targeting treatment of obesity, diabetes, and non-alcoholic steatohepatitis (NASH). At Obesity Week in November this year Boehringer Ingelheim presented data from the Phase 1b trial, demonstrating up to 13.7% weight loss and no unexpected safety findings following 16 weeks of dosing. Three parallel Phase 2 trials are ongoing. All subjects have been randomized in the first phase 2 trial which evaluates the dose-relationship of BI 456906 on HbA1c from baseline to 16 weeks relative to placebo in 410 people with diabetes (ClinicalTrials.gov Identifier: NCT04153929). Secondary objectives are to assess the effect on change in body weight and an open-label comparator (semaglutide) will allow for comparison of the effects against a pure GLP-1R agonist. The second Phase 2 randomized double-blind placebo-controlled dose-finding trial will evaluate BI 456906 in people with obesity or who are overweight with a BMI 27 kg/m2 or higher without diabetes (ClinicalTrials.gov Identifier: NCT04667377). Participants will receive a subcutaneous injection of either BI 456906 or placebo once a week for the duration of the trial. The primary endpoint of this trial is the percentage change in body weight at week 46 compared to placebo. The third Phase 2 randomized double-blind placebo-controlled dose-finding trial will evaluate BI 456906 in people with NASH and liver fibrosis (F2/F3) with and without diabetes (ClinicalTrials.gov Identifier: NCT04771273). The primary endpoint of this trial is the histological improvement of steatohepatitis without worsening of fibrosis after 48 weeks of treatment. Participants will receive a weekly subcutaneous injection of either different doses of BI 456906 or placebo for the duration of the trial. The program has received Fast Track designation from the U.S. FDA. Boehringer Ingelheim is funding all research, development and commercialization activities related to the treatment. Zealand Pharma is eligible to receive up to EUR 345 million in outstanding milestone payments, and high-single to low-double digit royalties on global sales. Inflammation Zealand Pharma is pursuing multiple pre-clinical programs in inflammatory diseases which will be detailed more as they progress through development. Complement inhibitors (with Alexion, AstraZeneca Rare Disease) Zealand Pharma and Alexion Pharmaceuticals announced in March 2019 that they will collaborate on the discovery and development of novel peptide therapies for complement-mediated diseases. Under the terms of the agreement, Alexion and Zealand Pharma entered into an exclusive collaboration for the discovery and development of subcutaneously delivered peptide therapies directed to up to four complement pathway targets. The lead program is a long-acting inhibitor of Complement C3 which has the potential to treat a broad range of complement mediated diseases. Zealand Pharma will lead the joint discovery and research efforts through the preclinical stage, and Alexion will lead development efforts beginning with IND filing and Phase 1 trials. We are looking to initiate a Phase 1 trial of the C3 inhibitor in 2022. For the lead target, Zealand Pharma is eligible to receive up to USD 610 million in development and sales milestone payments, plus royalties on global sales in the high single to low double digits. In addition, Alexion has the option to select up to three additional targets with Zealand Pharma eligible for USD 15 million upfront per target plus development/regulatory milestones for each target selected similar to the lead target with slightly reduced commercial milestones and royalties. Additional Updates On December 18, 2020 Amyndas Pharmaceuticals S.A. and Amyndas Pharmaceuticals LLC filed a complaint in the U.S. District Court for the District of Massachusetts, which named Alexion Pharmaceuticals, Inc., Zealand Pharma A/S and Zealand Pharma U.S., Inc. as defendants. The complaint alleges claims against Zealand Pharma A/S (and its U.S. subsidiary) and its collaboration partner Alexion Pharmaceuticals, Inc. (“Alexion”) related to Zealand Pharma A/S’s collaboration with Alexion on C3 peptide-based assets. The complaint alleges federal and state law claims, including claims for breach of confidentiality agreements, trade secret misappropriation and unfair competition. The complaint seeks an unspecified quantum of damages plus interest and injunctive relief. On June 8, 2021 the District Court dismissed the proceedings against Zealand Pharma U.S., Inc. for failure to state a claim, and dismissed the claims against Zealand Pharma A/S on the ground that the matter should be heard in the courts of Denmark. On July 6, 2021 Amyndas moved the District Court to reconsider its dismissal of the claims against Zealand Pharma A/S (and its U.S. subsidiary) and this was also dismissed. On 27 September 2021 Amyndas filed an appeal of this decision to the First Circuit Court of Appeals. It is also seeking an order from the District Court pursuant to Rule 54(b) to render the district court decision as final. Conference call today at 4 pm CEST / 10 am EDT Zealand Pharma’s management will host a conference call today at 4 pm CEST to present results through the first nine months of 2021. Participating in the call will be Chief Executive Officer Emmanuel Dulac, Chief Financial Officer Matt Dallas, and Chief Medical and Development Officer Adam Steensberg. The presentation will be followed by a Q&A session with the presenters as well as the President of Zealand Pharma U.S., Frank Sanders. The conference call will be conducted in English, and the dial-in numbers are: Denmark +45 32 720 417 United Kingdom +44 (0) 844 481 9752 United States +1 646 741 3167 France +33 (0) 170700781 Netherlands +31 (0) 207956614 Passcode 9606399 A live audio webcast of the call, including an accompanying slide presentation, will be accessible from the Investor section of Zealand Pharma’s website. Participants are advised to register for the webcast approximately 10 minutes before the start. A recording of the event will be available on the Investor section of Zealand Pharma’s website following the call. Upcoming events Zealand Pharma plans to publish results for the fourth quarter of 2021 on March 10, 2022. Total number of shares and voting rights in Zealand Pharma as of September 30, 2021 Number of shares (nominal value of DKK 1 each): 43,581,697 which is an increase of 39,859 from 43,428,192 as of June 30, 2021. Therefore, the current Share capital is (nominal value in DKK): 43,581,697. Number of voting rights: 43,581,697 About Zealand Pharma A/S Zealand Pharma A/S (Nasdaq: ZEAL) ("Zealand") is a biotechnology company focused on the discovery, development, and commercialization of peptide-based medicines. More than 10 drug candidates invented by Zealand have advanced into clinical development, of which two have reached the market. In addition, license collaborations with Boehringer Ingelheim and AstraZeneca create opportunity for more patients to potentially benefit from Zealand-invented peptide investigational agents currently in development. Zealand was founded in 1998 in Copenhagen, Denmark, and has presence throughout the U.S. that includes key locations in Boston, and Marlborough (MA). For more information about Zealand’s business and activities, please visit . Zegalogue® and V-Go® are registered trademarks of Zealand Pharma A/S. Safe Harbor / Forward-Looking Statements This press release contains “forward-looking statements”, as that term is defined in the Private Securities Litigation Reform Act of 1995, as amended, that provide Zealand Pharma’s expectations or forecasts of future events regarding the research, development and commercialization of pharmaceutical products and the company’s Financial Guidance for 2021. These forward-looking statements may be identified by words such as “aim,” “anticipate,” “believe,” “could,” “estimate,” “expect,” “forecast,” “goal,” “intend,” “may,” “plan,” “possible,” “potential,” “will,” “would” and other words and terms of similar meaning. You should not place undue reliance on these statements, or the scientific data presented. The reader is cautioned not to rely on these forward-looking statements. Such forward-looking statements are subject to risks, uncertainties and inaccurate assumptions, which may cause actual results to differ materially from expectations set forth herein and may cause any or all of such forward-looking statements to be incorrect, and which include, but are not limited to, unexpected costs or delays in clinical trials and other development activities due to adverse safety events or otherwise; unexpected concerns that may arise from additional data, analysis or results obtained during clinical trials; our ability to successfully market both new and existing products; changes in reimbursement rules and governmental laws and related interpretation thereof; government-mandated or market-driven price decreases for our products; introduction of competing products; production problems; unexpected growth in costs and expenses; our ability to integrate operate businesses in varying geographies; failure to protect and enforce our data, intellectual property and other proprietary rights and uncertainties relating to intellectual property claims and challenges; regulatory authorities may require additional information or further studies, or may reject, fail to approve or may delay approval of our drug candidates or expansion of product labeling; failure to obtain regulatory approvals in other jurisdictions; exposure to product liability and other claims; interest rate and currency exchange rate fluctuations; unexpected contract breaches or terminations; and the direct and indirect impacts of the ongoing COVID-19 pandemic on our business, results of operations and financial condition. If any or all of such forward-looking statements prove to be incorrect, our actual results could differ materially and adversely from those anticipated or implied by such statements. The foregoing sets forth many, but not all, of the factors that could cause actual results to differ from our expectations in any forward-looking statement. All such forward-looking statements speak only as of the date of this press release and are based on information available to Zealand Pharma as of the date of this release. We do not undertake to update any of these forward-looking statements to reflect events or circumstances that occur after the date hereof. Information concerning pharmaceuticals (including compounds under development) contained within this material is not intended as advertising or medical advice. NOTE: DKK/USD Exchange rates used: September 30, 2021 = 6.422 and September 30, 2020 = 6.3598 Maeve Conneighton Argot Partners Email: investors@zealandpharma.com For U.S. Media David Rosen Argot Partners Email: media@zealandpharma.com Key figures * DKK thousand Reviewed Audited INCOME STATEMENT AND COMPREHENSIVE INCOME Note Q3 2021 Q3 2020 Q1-Q3 2021 Q1-Q3 2020 FY 2020 Revenue 106,407 56,526 238,552 289,958 353,314 Gross margin 77,734 22,255 143,577 227,661 262,749 Research and development expenses -141,298 -139,332 -426,265 -430,991 -604,081 Sales and Marketing expenses -78,824 -97,429 -281,687 -172,282 -285,256 Administrative expenses -69,488 -40,567 -198,256 -111,232 -202,770 Net operating expenses -289,610 -277,328 -906,208 -714,505 -1,092,107 Other operating income 73 36,866 8 37,724 36,997 Operating result -211,803 -218,207 -762,623 -449,120 -792,361 Net financial items 16,071 -9,746 21,520 -18,749 -47,292 Result before tax -195,732 -227,953 -741,103 -467,869 -839,653 Income tax (1) -3,155 -621 3,216 1,686 -7,076 Net result for the period -198,887 -228,574 -737,887 -466,183 -846,729 Comprehensive result for the period -196,897 -225,841 -734,093 -463,777 -837,752 Earnings/loss per share – basic/diluted (DKK) -4.61 -5.76 -17.26 -12.39 -22.07 STATEMENT OF FINANCIAL POSITION September 30, 2021 September 30, 2020 December 31, 2020 Cash and cash equivalents 753,599 1,231,685 960,221 Marketable securities 295,379 296,909 297,345 Cash, cash equivalents and Marketable securities 1,048,978 1,528,594 1,257,566 Other assets 611,721 530,549 504,383 Total assets 1,660,699 2,059,143 1,761,949 Share capital ('000 shares) 43,582 39,779 39,800 Equity 1,185,746 1,590,003 1,229,311 Total liabilities 474,953 469,140 532,638 Equity ratio (2) 0.71 0.77 0.70 CASH FLOW Q1-Q3 2021 Q1-Q3 2020 FY 2020 Cash (used in)/provided by operating activities -923,731 -411,628 -688,716 Cash (used in)/provided by investing activities -6,255 -200,799 -196,807 Cash (used in)/provided by financing activities 705,914 780,826 760,941 Purchase of property, plant and equipment -5,854 -29,491 -25,044 Free cash flow (3) -929,585 -441,119 -713,760 OTHER September 30, 2021 September 30, 2020 December 31, 2020 Share price (DKK) 185.0 241.60 220.60 Market capitalization (MDKK) (4) 7,911 9,588 8,464 Equity per share (DKK) (5) 27.73 40.06 32.04 Average number of employees 345 322 297 Number of full-time employees at the end of the period 346 329 329 Notes: * The acquisition of the business from Valeritas is only reflected in key figures covering the period since April 2, 2020 being the acquisition date. (1) Zealand expects to be eligible to receive up to DKK 5.5 million in Danish corporate tax benefit related to R&D expenses incurred for 2021, of which DKK 4.1 million has been recognized for the period ended September 30, 2021, which is setoff against expected tax expense in the US. (2) Equity ratio is calculated as equity at the balance sheet date divided by total assets at the balance sheet date. (3) Free cash flow is calculated as the sum of cash flows from operating activities and purchase of property, plant and equipment. (4) Market capitalization is calculated as weighted outstanding shares at the balance sheet date times the share price at the balance sheet date. (5) Equity per share is calculated as shareholders' equity divided by weighted total number of ordinary shares less weighted treasury shares. Financial review The condensed interim financial statements are prepared in accordance with IAS 34 Interim Financial Reporting, as issued by the International Accounting Standards Board (IASB) and as adopted by the EU, and additional requirements of the Danish Financial Statements Act. The interim condensed consolidated financial statements are presented in DKK, which is also the functional currency of Zealand Pharma A/S (“the Company” or “the Group”). Comparative figures for the corresponding period in 2020 are shown in brackets except for the financial position, which expresses the comparative figures as of December 31, 2020. Financial results Revenue DKK thousand Q1-Q3 2021 Q1-Q3 2020 ∆ ∆ in percent Sale of goods 143,348 103,968 39,380 38% License and milestone revenue 95,204 185,990 -90,786 -49% Total revenue 238,552 289,958 -51,406 -18% Increase in revenue from the sale of goods is primarily attributable to the sales of V-Go and Zegalogue in 2021. Zegalogue became commercially available in June of 2021 and did not have any product revenue prior to that date. The product revenue from sale of goods of DKK 104.0 million in the table above is driven by activity for the six-month period April 2,2020 to September 30, 2020, following the Valeritas acquisition on April 2, 2020. The decrease in license and milestone revenue is mainly due to a milestone payment of DKK 149.1 million triggered and recognized as revenue in June 2020 from our partnership agreement with Boehringer Ingelheim. For further please see note 1. Gross margin DKK thousand Q1-Q3 2021 Q1-Q3 2020 ∆ ∆ in percent Gross margin 143,577 227,661 -84,084 -37% The decrease in gross margin is primarily due to the milestone payment of DKK 149.1 million triggered and recognized as revenue in June 2020 from our partnership agreement with Boehringer Ingelheim. This is partially offset by the increase in product revenue in the first nine months of 2021 related to the sales of Zegalogue and V-Go. Research and development expenses DKK thousand Q1-Q3 2021 Q1-Q3 2020 ∆ ∆ in percent Research and development expenses 426,265 430,991 -4,726 -1% Research and development expenses are primarily related to activities with our late-stage clinical programs for dasiglucagon and glepaglutide. Expenses are relatively flat between the periods. Sales and marketing expenses DKK thousand Q1-Q3 2021 Q1-Q3 2020 ∆ ∆ in percent Sales and marketing expenses 281,687 172,282 109,405 64% The increase in sales and marketing expenses is related to efforts for the Zegalogue launch as well as continued commercial support for the V-Go wearable insulin delivery device. The sales and marketing expenses of DKK 172.3 million in the table above are driven by activity for the six-month period April 2, 2020 to September 30, 2020, following the Valeritas acquisition on April 2, 2020. Administrative expenses DKK thousand Q1-Q3 2021 Q1-Q3 2020 ∆ ∆ in percent Administrative expenses 198,256 111,232 87,024 78% The increase in administrative expenses is costs related to the buildup and operations of the US subsidiary, which supports the commercial infrastructure as well as general and administrative purposes. Substantial US operations were acquired in April 2020 following the close of the Valeritas asset purchase agreement. Operating result DKK thousand Q1-Q3 2021 Q1-Q3 2020 ∆ ∆ in percent Operating result -762,623 -449,120 -313,503 -70% The operating result reflects gross margin, research and development expenses, sales and marketing and administrative expenses, as discussed above. Net financial items DKK thousand Q1-Q3 2021 Q1-Q3 2020 ∆ ∆ in percent Net financial items 21,520 -18,749 40,269 215% Financial income and financial expenses, which we refer to collectively as net financial items, consist of interest income and expense, fair market value adjustments, banking fees and impact from adjustments related to foreign exchange rates. The positive development from 2020 to 2021 is mainly related to the development in the DKK/USD exchange rate. Result before tax DKK thousand Q1-Q3 2021 Q1-Q3 2020 ∆ ∆ in percent Result before tax -741,103 -467,869 -273,234 -58% Result before tax reflects the operating result and net financial items, as discussed above. Income tax DKK thousand Q1-Q3 2021 Q1-Q3 2020 ∆ ∆ in percent Income tax 3,216 1,686 1,530 91% The net income tax (income) is mainly impacted by the tax deduction in Denmark, a prior period correction offset by tax expenses in US. No deferred tax asset regarding the Danish parent company has been recognized in the statement of financial position due to uncertainty as to whether tax losses carried forward can be utilized within the near term. Net result DKK thousand Q1-Q3 2021 Q1-Q3 2020 ∆ ∆ in percent Net result -737,887 -466,183 -271,704 -58% The decrease in the net result is primarily a result of increased sales and marketing and administrative expenses. The expense increase is due to the first nine months of 2020 only having commercial infrastructure effective April 2020 following the Valeritas asset purchase agreement and the company’s commercially launch of Zegalogue in June of 2021. In addition, there was a one-time milestone of DKK 149.1 million triggered in June 2020 from our partnership agreement with Boehringer Ingelheim. Liquidity and capital resources Equity DKK thousand September 30, 2021 December 31, 2020 ∆ ∆ in percent Equity 1,185,746 1,229,311 -43,565 -4% Equity ratio 71% 70% N/A N/A Equity ratio is calculated as equity at the balance sheet date divided by total assets at the balance sheet date. The increase in equity was mainly driven by a capital increase in January 2021 amounting to DKK 748.9 million offset by the loss for the period. Cash, cash equivalents and Marketable securities DKK thousand September 30, 2021 December 31, 2020 ∆ ∆ in percent Cash, cash equivalents and Marketable securities 1,048,978 1,257,566 -208,588 -17% The increase was mainly driven by the capital increase in January 2021 amounting to DKK 748.9 million offset by cash spent in the period. Cash flow DKK thousand Q1-Q3 2021 Q1-Q3 2020 ∆ ∆ in percent Cash used in operating activities -923,731 -411,628 -512,103 -124% Cash used in investing activities -6,255 -200,799 194,544 97% Cash from financing activities 705,914 780,826 -74,912 -10% Net cash flow -929,585 -441,119 -488,466 -111% The increase in cash used in operating activities from the same period in 2020 is mainly related to our sales and marketing and administrative expenses increasing as a result of the commercial activities and support for Zegalogue and the V-Go wearable insulin delivery device. In the first nine months of 2020 Zealand only had the sales and marketing expense and infrastructure effective April 2020 following the Valeritas asset purchase agreement. Cash used in investing activities in the first nine months of 2021 related mainly to acquisition of tangible assets. The investing activities in the first nine months of 2020 are mainly related to the acquisition of Valeritas. Cash from financing activities increased primarily because of the January 2021 financing with an aggregate gross amount of DKK 748.9 million. Cash from financing activities for the nine months ended September 30, 2020 was mainly related to June financing of gross DKK 657.7 million but also a private placement of gross DKK 137.2 million. This interim report contains forward-looking statements, including forecasts of future expenses as well as expected business-related events. Such statements are subject to risks and uncertainties as various factors, some of which are beyond the control of Zealand, may cause actual results and performance to differ materially from the forecasts made in this interim report. Without being exhaustive, such factors include e.g. the impact of the global COVID-19 pandemic, interest rate and currency exchange rate fluctuations, delay or failure of clinical trials and other development activities, production problems, unexpected contract breaches or terminations, government-mandated or market-driven price decreases for Zealand's products, introduction of competing products, Zealand's ability to successfully market both new and existing products, exposure to product liability and other lawsuits, changes in reimbursement rules and governmental laws and related interpretation thereof, unexpected growth in costs and expenses, and Zealand’s ability to integrate businesses in varying geographies with different commercial and operating characteristics. In particular, the global COVID-19 pandemic could potentially materially adversely impact our business and financial performance, including the timing of our clinical trials, projected regulatory approval timelines, our supply chain and sales of our approved products, as well as our Financial Guidance for 2021 in this interim report, particularly because the COVID-19 pandemic continues to evolve, and its breadth and significance on our business and financial performance is uncertain. A more extensive description of risk factors can be found in the 2020 Annual Report under the section Risk management and internal control. Management’s statement on the interim report The Board of Directors and the Management have considered and adopted the interim report of Zealand Pharma A/S for the three- and nine-month periods ended September 30, 2021. The condensed consolidated interim financial statements are prepared in accordance with IAS 34 Interim Financial Reporting, as issued by the International Accounting Standards Board (IASB) and as adopted by the EU, and additional requirements of the Danish Financial Statements Act. In our opinion, the interim report gives a true and fair view of the Group’s assets, equity and liabilities and financial position as of September 30, 2021 as well as of the results of the Group’s operations and cash flow for the period January 1 – September 30, 2021. Moreover, in our opinion, the Management’s Review gives a true and fair view of the development in the Company’s operations and financial conditions, of the net result for the periods and the financial position while also describing the most significant risks and uncertainty factors that may affect the Group. Copenhagen, November11, 2021 Management Emmanuel Dulac President and Chief Executive Officer Matthew Dallas Senior Vice President and Chief Financial Officer Adam Sinding Steensberg Executive Vice President and Chief Medical Officer Board of Directors Alf Gunnar Martin Nicklasson Chairman Kirsten Aarup Drejer Vice Chairman Jeffrey Berkowitz Board member Bernadette Mary Connaughton Board member Leonard Kruimer Board member Alain Munoz Board member Michael John Owen Board member Anneline Nansen Board member Employee elected Iben Louise Gjelstrup Board member Employee elected Jens Peter Stenvang Board member Employee elected Nikolaj Frederik Beck Board member Employee elected Independent auditor’s report To the shareholders of Zealand Pharma A/S We have reviewed the interim condensed consolidated financial statements of Zealand Pharma A/S for the three- and nine-month periods ended September 30, 2021, which comprise a condensed consolidated income statement and statement of comprehensive income for the three and nine-month periods ended September 30, 2021, statement of financial position at September 30, 2021, and statement of changes in equity and statement of cash flow, for the nine-month period ended September 30, 2021, and notes. The interim condensed consolidated financial statements are prepared in accordance with IAS 34 Interim Financial Reporting, as issued by the International Accounting Standards Board (IASB) and as adopted by the EU, and additional requirements of the Danish Financial Statements Act. Management's responsibilities for the interim condensed consolidated financial statements Management is responsible for the preparation of interim condensed consolidated financial statements in accordance with IAS 34 Interim Financial Reporting, as issued by the International Accounting Standards Board (IASB) and as adopted by the EU, and additional requirements of the Danish Financial Statements Act and for such internal control as Management determines is necessary to enable the preparation of interim condensed consolidated financial statements that are free from material misstatement, whether due to fraud or error. Auditor's responsibilities Our responsibility is to express a conclusion on the interim condensed consolidated financial statements. We conducted our review in accordance with the International Standard on Review of Interim Financial Information Performed by the Independent Auditor of the Entity and additional requirements applicable in Denmark. This requires us to conclude whether anything has come to our attention that causes us to believe that the interim condensed consolidated financial statements, taken as a whole, are not prepared, in all material respects, in accordance with IAS 34 Interim Financial Reporting, as issued by the International Accounting Standards Board (IASB) and as adopted by the EU, and additional requirements of the Danish Financial Statements Act. This standard also requires us to comply with ethical requirements. A review of the interim condensed consolidated financial statements in accordance with the International Standard on Review of Interim Financial Information Performed by the Independent Auditor of the Entity is a limited assurance engagement. The auditor performs procedures primarily consisting of making enquiries of Management and others within the company, as appropriate, applying analytical procedures and evaluate the evidence obtained. The procedures performed in a review are substantially less that those performed in an audit conducted in accordance with the International Standards on Auditing. Accordingly, we do not express an audit opinion on the interim condensed consolidated financial statements. Conclusion Based on our review, nothing has come to our attention that causes us to believe that these interim condensed consolidated financial statements are not prepared, in all material respects, in accordance with IAS 34 Interim Financial Reporting, as issued by the International Accounting Standards Board (IASB) and as adopted by the EU, and additional requirements of the Danish Financial Statements Act. Copenhagen, November11, 2021 EY Godkendt Revisionspartnerselskab CVR no. 30 70 02 28 Christian Schwenn Johansen State Authorized Public Accountant mne33234 Rasmus Bloch Jespersen State Authorized Public Accountant mne35503 Interim condensed consolidated financial statements Interim condensed consolidated income statement for the three- and nine-month periods ended September 30, 2021 and 2020. Reviewed DKK thousand Note Q3 2021 Q3 2020 YTD 2021 YTD 2020 Revenue 2 106,407 56,526 238,552 289,958 Cost of goods sold -28,743 -34,271 -84,005 -62,297 Royalty expenses 3 70 0 -10,970 0 Gross margin 77,734 22,255 143,577 227,661 Research and development expenses 4 -141,298 -139,332 -426,265 -430,991 Sales and marketing expenses 4 -78,824 -97,429 -281,687 -172,282 Administrative expenses 4 -69,488 -40,567 -198,256 -111,232 Total Operating expenses -289,610 -277,328 -906,208 -714,505 Other operating income 5 73 36,866 8 37,724 Operating result -211,803 -218,207 -762,623 -449,120 Financial income 6 18,548 3,799 29,456 900 Financial expenses 7 -2,477 -13,545 -7,936 -19,649 Result before tax -195,732 -227,953 -741,103 -467,869 Income tax 8 -3,155 -621 3,216 1,686 Net result for the period -198,887 -228,574 -737,887 -466,183 Earnings/loss per share – basic/diluted (DKK) 9 -4.61 -5.76 -17.26 -12.39 Interim condensed consolidated statement of comprehensive income (loss) for the three- and nine-month periods ended September 30, 2021 and 2020. Reviewed DKK thousand Note Q3 2021 Q3 2020 YTD 2021 YTD 2020 Net result for the period -198,887 -228,574 -737,887 -466,183 Adjustment of foreign currency fluctuations on subsidiaries 1,990 2,733 3,794 2,405 Comprehensive result for the period -196,897 -225,841 -734,093 -463,777 Interim condensed consolidated statements of cash flow for the nine month periods ended September 30, 2021 and 2020 and for the twelve month period ended December 31, 2020 Reviewed Audited DKK thousand Note YTD 2021 YTD 2020 FY 2020 Net result for the period -737,887 -466,183 -846,729 Bargain purchase 0 -36,692 -36,395 Adjustments for other non-cash items -20,463 87,607 143,138 Change in working capital -96,219 43,133 97,818 Interest received 0 901 895 Interest paid -5,347 -5,862 -4,562 Deferred revenue 2 -16,844 -34,532 -42,881 Income tax paid/received 8 -46,971 0 0 Cash used in operating activities -923,731 -411,628 -688,716 Acquisition of Valeritas business, net of cash acquired 0 -167,725 -167,791 Change in deposits -401 -3,583 -3,972 Purchase of property, plant and equipment 11 -5,854 -29,491 -25,044 Cash used in investing activities -6,255 -200,799 -196,807 Proceeds from issuance of shares related to exercise of share-based compensation 19 21,149 38,832 41,363 Proceeds from issuance of shares 19 748,975 794,929 791,503 Costs related to issuance of shares -46,894 -42,689 -42,706 Purchase of treasury shares -28,595 0 0 Leasing liabilities 12 11,279 -10,246 -29,219 Cash from financing activities 705,914 780,826 760,941 Decrease/increase in cash and cash equivalents -224,072 168,399 -124,582 Cash and cash equivalents at beginning of period 960,221 1,081,060 1,081,060 Exchange rate adjustments 17,450 -17,775 3,743 Cash and cash equivalents at end of period 753,599 1,231,685 960,221 Interim condensed consolidated statements of financial position as of September 30, 2021 and December 31, 2020 Reviewed Audited DKK thousand Note September 30, 2021 December 31, 2020 ASSETS Non-current assets Intangible assets 10 54,710 57,485 Property, plant and equipment 11 83,308 85,040 Right-of-use assets 12 136,964 127,998 Deposits 17,051 16,650 Corporate tax receivable 1,268 1,268 Prepaid expenses 10,491 13,117 Deferred tax assets 8,344 8,370 Other investments 13 34,682 32,333 Total non-current assets 346,818 342,261 Current assets Inventories 14 116,359 65,040 Trade receivables 15 80,489 46,484 Prepaid expenses 16 30,469 35,156 Corporate tax receivable 8 25,442 5,500 Other receivables 17 12,144 9,942 Marketable securities 13 295,379 297,345 Cash and cash equivalents 18 753,599 960,221 Total current assets 1,313,881 1,419,688 Total assets 1,660,699 1,761,949 EQUITY AND LIABILITIES Share capital 19 43,582 39,800 Share premium 20 4,157,533 3,470,787 Translation reserve 12,771 8,977 Accumulated loss -3,028,140 -2,290,253 Equity 1,185,746 1,229,311 Deferred revenue 19,525 44,587 Other liabilities 18,227 16,744 Lease liabilities 12 126,505 116,047 Non-current liabilities 164,257 177,378 Trade payables 21 54,908 70,384 Corporate tax payables 0 30,394 Lease liabilities 12 15,527 14,072 Deferred revenue 61,401 53,182 Discount and rebate liabilities 27,310 36,673 Other liabilities 22 151,550 150,555 Current liabilities 310,696 355,260 Total liabilities 474,953 532,638 Total equity and liabilities 1,660,699 1,761,949 Interim condensed consolidated statements of changes in equity for the nine-month period ended September 30, 2021 and 2020 Reviewed DKK thousand Share Share Translation Retained capital premium reserve loss Total Equity at January 1, 2020 36,055 2,650,142 0 -1,443,524 1,242,673 Other comprehensive income for the period 0 0 2,405 0 2,405 Net loss for the period 0 0 0 -466,183 -466,183 Share-based compensation 0 20,037 0 0 20,037 Capital increase, see note 19 3,724 830,037 0 0 833,761 Costs related to capital increases 0 -42,689 0 0 -42,689 Equity at September 30, 2020 39,779 3,457,526 2,405 -1,909,707 1,590,003 Equity at January 1, 2021 39,800 3,470,787 8,977 -2,290,253 1,229,311 Other comprehensive income for the period 0 0 3,794 0 3,794 Net loss for the period 0 0 0 -737,887 -737,887 Share-based compensation 0 37,492 0 0 37,492 Treasury shares, see note 20 0 -70,195 0 0 -70,195 Capital increase, see note 19 3,782 766,342 0 0 770,124 Costs related to capital increases 0 -46,893 0 0 -46,893 Equity at September 30, 2021 43,582 4,157,533 12,771 -3,028,140 1,185,746 Note 1 - Basis of preparation and changes to the Group’s accounting policies Basis of preparation The interim condensed consolidated financial statements of Zealand Pharma A/S have been prepared in accordance with IAS 34, Interim Financial Reporting, as issued by the International Accounting Standards Board (IASB) and as adopted by EU and additional requirements of the Danish Financial Statements Act. The interim condensed consolidated financial statements are presented in Danish kroner (DKK) which is also the functional currency of the parent company. The accounting policies used in the interim condensed consolidated financial statements are consistent with those used in the Company’s annual financial statement for the year ended December 31, 2020. New standards, interpretations and amendments adopted by the Group A few amendments apply for the first time in 2021, but do not have an impact on the interim condensed consolidated financial statements of the Group. Significant judgements and estimates In the preparation of the interim condensed consolidated financial statements, the Company’s management (“Management”) makes several accounting estimates that form the basis for the presentation, recognition and measurement of the Company’s assets and liabilities. In the application of the Company’s accounting policies, Management is required to make judgments, estimates and assumptions about the carrying amounts of assets and liabilities that are not readily apparent from other sources. The estimates and associated assumptions are based on historical experience and other factors that are considered to be relevant. Actual results may differ from these estimates. The estimates and underlying assumptions are reviewed on an ongoing basis. Revisions to accounting estimates are recognized in the period in which the estimate is revised if the revision affects only that period, or in the period of the revision and future periods if the revision affects both current and future periods. The estimates used are based on assumptions assessed as reasonable by Management; however, estimates are inherently uncertain and unpredictable. The assumptions can be incomplete or inaccurate, and unexpected events or circumstances might occur. Furthermore, the Company is subject to risks and uncertainties that might result in deviations in actual results compared with estimates. For further information regarding significant accounting estimates and judgments see note 1 in the Annual Report for 2020. Information on COVID-19 Our business, operations and clinical studies were, of course, impacted by the effects of COVID-19. Although our clinical studies continued without interruption during 2021 and 2020, there were delays and increased total costs arising from the implications of COVID-19. However, we have not recognized any write-offs, impairments of assets, or losses to onerous contracts due to COVID-19. The COVID-19 pandemic is also having an effect on other aspects of our business, including: our third-party manufacturers, and other third parties; albeit with no material effect or impact. The COVID-19 pandemic may, in the long-term, affect the productivity of our staff; our ability to attract, integrate, manage and retain qualified personnel or key employees; our global supply chains and relationships with vendors and other parties; significant disruption of global financial markets; and reduced ability to secure additional funding. We continuously monitor the COVID-19 pandemic and its potential impact on our business and financials. Note 2 - Revenue Revenue can be specified as follows: DKK thousand Q3 2021 Q3 2020 Q1-Q3 2021 Q1-Q3 2020 Alexion Pharmaceuticals Inc. 6,497 10,645 16,844 36,870 Sanofi-Aventis Deutschland GmbH 0 0 30,669 0 Boehringer Ingelheim International GmbH 22,311 0 22,311 149,120 Protagonist Therapeutics Inc. 25,380 0 25,380 0 Total license and milestone revenue 54,188 10,645 95,204 185,990 Total sale of goods revenue net 52,219 45,881 143,348 103,968 Total revenue 106,407 56,526 238,552 289,958 Total revenue recognized over time 6,497 10,645 16,844 36,870 Total revenue recognized at a point in time 99,910 45,881 221,708 253,088 License revenue for the first nine months of 2021 of DKK 16.8 million is related to the research and development agreement with Alexion Pharmaceuticals entered into in March 2019. Under the agreement DKK 80.9 million is accounted for as deferred revenue as of September 30, 2021. License and milestone revenue for the first nine months of 2021 is DKK 95.2 million of which DKK 30.7 million is related to the Sanofi agreement. Zealand is still eligible for a payment from Sanofi of up to USD $10.0 million which is expected in 2022. On August 4, 2021 Zealand Pharma and Protagonist Therapeutics Inc reached a mutually acceptable settlement of the proceedings and the agreement will continue between the parties on agreed terms. Protagonist paid the sum of USD 2.5 million as the first payment under the settlement agreement and will pay an additional payment of USD 1.5 million in 2022. The complete settlement of USD 4.0 million has been recognized as revenue in Q3 2021. Sale of goods revenue for the first nine months of 2021 of DKK 138.9 million related to V-Go and DKK 4.4 million related to Zegalogue. The net sales comprise of gross sales of DKK 270.3 million and discounts and rebates of DKK -127.0 million. Zealand is managed and operated as one business unit, which is reflected in the organizational structure and internal reporting. No separate lines of business or separate business entities have been identified with respect to any of the product candidates or geographical markets and no segment information is currently included in the internal reporting. Net revenue in the US for the nine-month period ended September 30, 2021 is DKK 185.6 million including license and milestone revenue and sale of goods. Net sales in Germany amounts to DKK 53.0 million including license and milestone revenue. No other country accounts for more than 10% of the net total sales. Note 3 – Royalty expense Royalty expense relates to cost to 3rd parties in connection with the Sanofi royalty agreement. Note 4 – Research and development, Sales and marketing and Administrative expenses The increase September YTD 2021 over 2020 is mainly due to the acquisition of Valeritas in April 2020, which is impacting Sales and marketing and Administrative expenses, and launch of Zegalogue in Q2 2021, impacting Sales and marketing expenses. Note 5 – Other operating income Recognized other operating income can be specified as follows: DKK thousand Q3 2021 Q3 2020 Q1-Q3 2021 Q1-Q3 2020 Gain from bargain purchase 0 36,692 0 36,692 Other 73 174 8 1,032 Other operating income 73 36,866 8 37,724 The bargain purchase recognized in 2020 is related to Valeritas business acquisition in April 2020. Note 6 – Financial income Recognized financial income can be specified as follows: DKK thousand Q3 2021 Q3 2020 Q1-Q3 2021 Q1-Q3 2020 Currency exchange adjustments 17,938 0 29,427 0 Fair value adjustment 602 3,799 0 0 Other 8 0 29 900 Financial income 18,548 3,799 29,456 900 Note 7 - Financial expenses Recognized financial expenses can be specified as follows: DKK thousand Q3 2021 Q3 2020 Q1-Q3 2021 Q1-Q3 2020 Interest expenses and banking fees -2,477 -2,257 -6,358 -5,862 Fair value adjustment 0* 0* -1,578 -2,535 Currency exchange adjustments 0 -11,288 0 -11,252 Financial expenses -2,477 -13,545 -7,936 -19,649 *Fair value adjustments for the 3 months period in Q3 2020and 2021 is a net income and therefore not included in the table as it is recognized under financial income in note 6. Note 8 – Income tax The tax amount recognized in 2021 includes tax income of DKK 4.1 million relating to corporate tax benefit in Denmark, a correction in US to prior periods of DKK 7.3 million offset by tax expenses in US of DKK 8.2 million. Income taxes paid in 2021 include payable tax from 2020 and payments on account for 2021 for our US activities. For 2020 no income taxes have been paid. Note 9 - Earnings/Loss per share The earnings/loss and weighted average number of ordinary shares used in the calculation of basic and diluted earnings/loss per share are as follows: DKK thousand Q3 2021 Q3 2020 Q1-Q3 2021 Q1-Q3 2020 Net earnings/loss for the period -198,887 -228,574 -737,887 -466,183 Net earnings/loss used in the calculation of basic earnings/loss per share -198,887 -228,574 -737,887 -466,183 Weighted average number of ordinary shares 43,552,583 39,750,959 43,053,532 37,701,621 Weighted average number of treasury shares -401,599 -64,223 -290,847 -64,223 Weighted average number of ordinary shares used in the calculation of basic/diluted loss per share 43,150,984 39,686,736 42,762,685 37,637,398 Earnings/loss per share – basic/diluted (DKK) -4.61 -5.76 -17.26 -12.39 The following potential ordinary shares are anti-dilutive and are therefore excluded from the weighted average number of ordinary shares for the purpose of diluted earnings/loss per share: September 30, 2021 September 30, 2020 Outstanding warrants under the 2015 Employee incentive program 1,526,779 1,941,209 Outstanding warrants under the 2020 Employee incentive program 63,217 63,217 Outstanding Performance Share Units (PSUs) under the LTIP 2019 program 19,765 19,765 Outstanding Restricted Share Units (RSUs) under the LTIP 2020 program 27,466 27,466 Outstanding Performance Share Units (PSUs) under the LTIP 2021 program 282,852 0 Outstanding Restricted Share Units (RSUs) under the LTIP 2021 program 452,450 0 Total outstanding warrants/PSUs/RSUs 2,372,529 2,051,657 For further information on the Employee incentive programs please see note 6 in the Annual Report for 2020. Note 10 – Intangible assets Intangible assets of DKK 54.7 million recognized as of September 30, 2021 compared to DKK 57.5 million as of December 31, 2020. The decrease is primarily due to currency translation and amortization. The majority of the intangible assets occurred from the business combination of Valeritas activities on April 2, 2020. Since the 12 months period has expired all amounts are assessed to be final and no further adjustments relating to the business combination can occur. Note 11 – Property, plant and equipment DKK thousand September 30, 2021 December 31, 2020 Plant and machinery 36,665 41,911 Other fixtures and fittings 7,173 8,337 Building improvements 31,094 31,769 Assets under construction 8,376 3,023 Carrying amount 83,308 85,040 The decrease from DKK 85.0 million at December 31, 2020 to DKK 83.3 million as at September 30, 2021 is primarily related to depreciations in the period, partially offset by an increase in assets under construction in Denmark. Note 12 - Right of use assets and lease liabilities Right-of-use-assets of DKK 137.0 million and lease liability of DKK 142.0 million were recognized as at September 30, 2021 as compared to DKK 128.0 million and DKK 130.1 million, respectively, as of December 31, 2020. The increase is primarily related to currency translation and new smaller additions, offset by depreciations and payments. Note 13 - Financial instruments As of September 30, 2021, and December 31, 2020, the following financial instruments are measured at fair value through profit or loss: DKK thousand September 30, 2021 December 31, 2020 Marketable securities 295,379 297,345 Other investments 34,682 32,333 Financial assets measured at fair value 330,061 329,678 The fair value of marketable securities and other investments is based on Level 1 and Level 3, respectively, in the fair value hierarchy. No financial assets are based on Level 2. Other investments consist of a USD 5.4 million (December 31, 2020: USD 5.4 million) investment in Beta Bionics, Inc., the developer of iLet™, a fully integrated dual-hormone pump (bionic pancreas) for autonomous diabetes care. In determining fair value, Zealand considered the impact of any recent share capital issuances by Beta Bionics as an indicator of the fair value of the shares. In particular, Beta Bionics undertook a capital offering in June 2019 and subsequent infliction points was used as the basis for determining fair value. Measurement is considered a level 3 measurement. A net fair value adjustment of DKK 1.6 million from marketable securities and other investments have been recognized in financial expenses, as of September 30, 2021 (September 30, 2020: DKK 2.5 million in financial expense). There were no transfers between levels 1, 2 and 3 for recurring fair value measurements during the interim periods ended September 30, 2021 and 2020. Note 14 - Inventories DKK thousand September 30, 2021 December 31, 2020 Raw materials 35,487 14,398 Work in progress 29,250 13,723 Finished goods 51,622 36,919 Inventories 116,359 65,040 In 2021 a reversal of previous periods estimated write-down on prelaunch inventory with a net positive income statement effect of DKK 27.0 million on research and development expenses was recognized, as a consequence of our FDA approval of Zegalogue in March 2021. Note 15 – Trade receivables The increase in Trade receivables from DKK 46.5 million at December 31, 2020 to DKK 80.5 million at September 30, 2021 is mainly related to extended payment terms with our larger customers, DKK 9.6 million receivable from Protagonist Therapeutics Inc. and increase in the USD/DKK exchange rate. Note 16 – Prepaid expenses The decrease in Prepaid expenses from DKK 48.3 million at December 31, 2020 to DKK 41.0 million at September 30, 2021 is mainly relating to timing differences. Note 17 - Other receivables DKK thousand September 30, 2021 December 31, 2020 VAT 1,646 3,887 Other 10,498 6,055 Other receivables 12,144 9,942 Other receivables are mainly related to various receivables including VAT receivables, sub-supplier arrangements etc. Note 18 - Cash and cash equivalents DKK thousand September 30, 2021 December 31, 2020 DKK 228,654 286,222 USD 505,671 568,444 EUR 19,274 105,555 Total cash and cash equivalents 753,599 960,221 Note 19 - Changes in share capital The following changes have occurred in the share capital during the respective year-to-date interim periods: No. of shares (thousand) Share capital at January 1, 2020 36,055 Increase due to issue of new shares 3,724 Share capital at September 30, 2020 39,779 Share capital at January 1, 2021 39,800 Increase due to issue of new shares 3,782 Share capital at September 30, 2021 43,582 Note 20 – Treasury shares As part of the capital increase made in January 2021, a total of 200,000 shares were purchased through a share swap transaction. As the payment is not due yet, a liability of DKK 41.6 million is recognized under other liabilities. Refer to note 22. Further in June and July 2021, the Company acquired owns shares through a share buyback program. The total number of shares acquired was 154,187 of a total value of DKK 28,597,426. The total number of treasury shares as of September 30, 2021 is 418,410 and will be used for long term incentive compensation plans. Note 21 – Trade payables The decrease in Trade payables from DKK 70.4 million as of December 31, 2020 to DKK 54,9 million at September 30, 2021 is mainly relating to timing differences. Note 22 - Other liabilities DKK thousand September 30, 2021 December 31, 2020 Employee benefits 57,360 101,028 Royalty payable to third party 5,715 5,732 Treasury shares purchase – See note 20 41,600 0 CRO liabilities 12,510 28,267 Other payables 34,365 32,272 Total other liabilities 151,550 167,299 The decrease in other liabilities is mainly related to a decrease in Employee benefits due to the payout of bonus in Q1 2021 and lower CRO liabilities, offset by a one-off liability regarding purchase of treasury shares of DKK 41.6 million. Other payables comprise of payables to authorities etc. Note 23 - Contingent assets, liabilities and contractual obligations Contingent assets As of September 30, 2021, Zealand is still eligible for a payment from Sanofi of up to USD 10.0 million which is expected in 2022. However, it is Management’s opinion that the amount of any payment cannot be determined on a sufficiently reliable basis, and therefore the company has not recognized an asset in the statement of financial position of the Group. Contingent liabilities and contractual obligations As of September 30, 2021, total contractual obligations related to agreements with CRO’s and CMO’s amounted to DKK 320.5 million (DKK 56.2 million for 2021 and DKK 264.4 million for the years 2022 up to and including 2025). Zealand may be required to pay future development, regulatory and commercial milestones related to the acquisition of Encycle Therapeutics. Refer to note 13 in the 2020 Annual Report. Note 24 - Significant events after the reporting period The Company has November 5, 2021 announced a collaboration agreement with DEKA Research & Development Corp. For further information, please see the company announcement no. 66/2021 on The Company’s website. 1 Castellanos, L.E. et al. (2021). Performance of the insulin-only iLet bionic pancreas and the bihormonal iLet using dasiglucagon in adults with type 1 diabetes in a home-use setting. Diabetes Care, 44:e118–e120. 2 Russell, S. et al. (2020). Performance of the bihormonal iLet bionic pancreas with the stable glucagon analog dasiglucagon. [ePoster]. ATTD.

Company annoucement - No. 50 / 2021 Interim report for H1 2021 Zealand Pharma Launches ZEGALOGUE® (dasiglucagon) Injection and Advances Pipeline Programs Across Multiple Therapeutic Areas Copenhagen, DK and Boston, MA, U.S. August 12, 2021 – Zealand Pharma A/S (Nasdaq: ZEAL) (CVR-no. 20045078) a biotechnology company focused on the discovery, development and commercialization of innovative peptide-based medicines, today announced financial results for the first half of 2021. Financial results for the first half of 2021 Business highlights for the first half of 2021 Emmanuel Dulac, President and Chief Executive Officer at Zealand Pharma, comments: “It has been an exciting first half of the year at Zealand Pharma as we continue to execute our mission to use our innovative peptide platform to address unmet needs in metabolic and gastrointestinal diseases. We are pleased with the U.S. FDA approval and commercial launch of ZEGALOGUE, which not only has the potential to help people with diabetes feel more confident and prepared to handle severe hypoglycemic episodes, but also represents the transformation of our company to a fully integrated biopharmaceutical company. We look forward to building upon the momentum of the launch as our commercial team brings ZEGALOGUE to patients in need and we continue to advance our other pipeline programs. We have made notable progress across our pipeline in the first half of the year, and with multiple clinical milestones on the horizon in the second half of the year, we are well positioned to achieve our goal of offering five marketed products by 2025.” Financial guidance for 2021 There is no change from the financial guidance issued on March 11, 2021. Net product revenue from the sales of commercial products is expected to be DKK 220 million +/- 10%. In 2021, Zealand Pharma expects revenue from existing license agreements. However, since such revenue is uncertain in terms of size and timing, Zealand Pharma does not intend to provide guidance on such revenue. Net operating expenses in 2021 are expected to be DKK 1,250 million +/-10%. Update regarding COVID-19 Zealand Pharma continues to monitor the COVID-19 pandemic and take precautions to keep our employees, patients, business and clinical partners safe. This is an ongoing exercise in monitoring the effects of the pandemic on all of our key stakeholders and responding appropriately. We maintain compliance with guidance from applicable government and health authorities as appropriate. We have adapted the way we work to support our community’s efforts to reduce the transmission of COVID-19 and protect our employees, while continuing to provide patient care and maintain business continuity. Zealand Pharma has taken measures to secure its discovery activities, which remain ongoing, while work in laboratories and offices has been organized to reduce the risk of COVID-19 transmission. The impact of COVID-19 on our research activities has thus far been minimal. Employees who can work from home have been doing so, while those needing to work in laboratory facilities are divided into shifts to reduce the number of people gathered at one time. Business travel has been minimized and online and video conference technology is used to meet virtually rather than in person. We have continued our clinical trials while working with authorities, investigators, trial sites and contract research organizations to minimize site visits and ensure optimal trial follow-up. In late April, Zealand Pharma in Denmark commenced a gradual return to office program, which currently allows up to 60% occupancy, consistent with local health authority guidelines and practice including frequent testing, sanitizing, and other protective measures. COVID-19 restrictions have not affected our phase 3-program for dasiglucagon in congenital hyperinsulinism (CHI) and we expect topline data from the second Phase 3 trial in 2021. The pandemic impacted the speed of patient recruitment for our Phase 3 trial with glepaglutide for the treatment of short bowel syndrome, and results are expected in 2022. We currently do not anticipate changes to the timelines for the bi-hormonal artificial pancreas pump Phase 3 program. Our research and in particular our development programs may be impacted if the pandemic continues to put increased pressure on hospital systems, slow recruitment of patients into the trials or cause lockdowns that affect our clinical trial sites if key external medical resources are diverted elsewhere. Direct engagement with health care providers and patients has been reduced and transformed by leveraging virtual meetings, training, and support. Our commercial team is focused on supporting the businesses for the V-Go® wearable insulin delivery device and Zegalogue while ensuring a continued high level of service and support for existing patients. Product Launch Update Dasiglucagon is a stable glucagon analog commercially available as Zegalogue® (dasiglucagon) injection, and indicated for the treatment of severe hypoglycemia in people with diabetes aged 6 and over, and being developed in three additional distinct indications: Zegalogue® (dasiglucagon) injection for severe hypoglycemia Zegalogue (dasiglucagon) injection was approved by the U.S. FDA on March 22, 2021 for the treatment of severe hypoglycemia in people with diabetes aged 6 and over. Zegalogue is available in both an auto injector and a prefilled syringe. The approval was based on efficacy results from three pivotal trials in adults and children with type 1 diabetes, whereby the primary endpoint of time to plasma glucose recovery, was successfully achieved with a median time to blood glucose recovery of 10 minutes following Zegalogue administration. In these Phase 3 studies, the most common adverse events reported (≥2%) were nausea, vomiting, headache, diarrhea, and injection site pain in adults; and nausea, vomiting, headache and injection site pain in pediatric patients. Zegalogue launched in the U.S. in late June 2021. Pipeline Update Type 1 Diabetes Management Dasiglucagon for bi-hormonal artificial pancreas pumps Zealand Pharma is developing a 1 ml cartridge containing 4 mg/ml dasiglucagon, intended for use in bi-hormonal artificial pancreas pumps. Zealand is collaborating with Beta Bionics, developer of the iLet™, a pocket-sized, dual-chamber, autonomous, glycemic control system. The iLet mimics a biological pancreas by calculating and dosing insulin and/or glucagon (dasiglucagon) as needed, based on data from the diabetic person’s continuous glucose monitor. Top-line results from a Phase 2 trial in patients with type 1 diabetes showed that the bi-hormonal iLet using dasiglucagon provided increased glycemic control over the insulin-only iLet. During the bi-hormonal period, 90% of participants had a mean CGM glucose level of < 154 mg/dL, whereas only 50% of participants on the insulin-only iLet achieved this. Importantly these glycemic targets were achieved while time with blood glucose levels < 54 mg/dL was only 0.3% in the bi-hormonal and 0.6% in the insulin-only arm. An in-use study of dasiglucagon in the iLet bihormonal bionic pancreas has been successfully completed supporting the overall clinical development program and Zealand partner Beta Bionics is on track to begin test run screening for the iLet bihormonal pivotal Phase 3 trial, utilizing dasiglucagon and insulin, by the end of 2021. Approximately 350 adults and 350 children with type 1 diabetes will be randomized into the Phase 3 trial. The primary outcome measure is superiority on HbA1c of the bihormonal iLet configuration using dasiglucagon over the insulin only iLet configuration at week 26. Overall, the program has been designed to demonstrate the clinical outcome of utilizing dasiglucagon in the bihormonal iLet versus an insulin-only iLet, while also comparing these results to intensified usual care. Dasiglucagon mini-dose pen Zealand is developing a dasiglucagon mini-dose pen for potential treatment of exercise-induced hypoglycemia in people living with type 1 Diabetes and for people who suffer from meal-induced hypoglycemia following gastric bypass surgery. Trials conducted in hospital settings have shown the clinical potential for using low doses of dasiglucagon to correct moderate hypoglycemia. Top-line results from a Phase 2a dose-finding trial in people with type 1 diabetes were presented at the American Diabetes Association congress in June 2021. In addition, top-line results of a post bariatric hypoglycemia Phase 2a dose-finding clinical proof of concept trial were reported in 2020 and showed that mini doses of dasiglucagon reduced meal-induced hypoglycemia compared to placebo in individuals who had undergone gastric bypass bariatric surgery. Out-patient Phase 2 trials in exercise-induced hypoglycemia in people living with type 1 diabetes and for people that suffer from meal-induced hypoglycemia following gastric bypass surgery were initiated in the second quarter this year (ClinicalTrials.gov Identifier: NCT04764968 and NCT04836273) and the first subjects have been enrolled into the out-patient Phase 2 trial evaluating effectiveness of pen-administered low-dose dasiglucagon for prevention and treatment of hypoglycemia in people with type 1 diabetes. Rare Diseases Dasiglucagon for congenital hyperinsulinism (CHI) The potential for chronic dasiglucagon infusion delivered via a pump to prevent hypoglycemia in children with CHI is being evaluated in a Phase 3 program. The aim is to reduce or eliminate the need for intensive hospital treatment, reduce the frequency of dangerous low blood glucose and need for constant feeding, and to potentially delay or eliminate the need for pancreatectomy. The FDA and the European Commission both granted orphan drug designation to dasiglucagon for the treatment of CHI. We announced data from the first Phase 3 trial in the program, trial 17109, in December 2020. This trial evaluated children from 3 months to 12 years old with more than three hypoglycemic events per week despite previous near-total pancreatectomy and/or maximum medical therapy. Dasiglucagon on top of standard of care (SOC) did not significantly reduce the rate of hypoglycemia compared to SOC alone when assessed by the primary endpoint, intermittent self-measured plasma glucose. However, hypoglycemia was reduced by 40–50% with dasiglucagon as compared to SOC alone when assessed by blinded continuous glucose monitoring. Dasiglucagon treatment was assessed to be well tolerated in the study and 31 out of 32 patients continued into the long-term extension study. We are conducting additional analyses and engaging with regulatory authorities to discuss the results of 17109 while awaiting the outcome of a second Phase 3 trial, 17103, in neonates up to 12 months old with CHI. Results from the 17103 trial are expected in 2021. Glepaglutide Zealand Pharma is developing treatments for gastrointestinal diseases, with a current focus on short bowel syndrome (SBS). One of the leading programs in Zealand Pharma’s pipeline is glepaglutide, a long-acting GLP-2 analog being developed in an auto-injector with potential for convenient weekly administration. EASE-SBS 1 is the pivotal Phase 3 trial with a planned enrolment of 129 patients with SBS that seeks to establish the efficacy and safety of once- and twice-weekly administration of glepaglutide. They will be treated for six months whereafter they are offered a further 2+2-year treatment with glepaglutide in the extension trials, EASE-SBS 2 and 3. The primary endpoint is the absolute reduction in parenteral support achieved by the end of the trial, with results expected in 2022. The U.S. FDA granted orphan drug designation to glepaglutide for the treatment of SBS. Dapiglutide Dapiglutide (pINN) is a long-acting GLP-1R/GLP-2R dual agonist. The Phase 1a single-ascending dose, safety and tolerability trial investigating dapaglutide in healthy volunteers was completed in Q3 2020 and dapiglutide was found to have an acceptable safety and tolerability profile. Results showed a plasma half-life allowing for once weekly dosing. Based on the results of the Phase 1a trial, Zealand initiated a Phase 1b (multiple ascending dose) safety and tolerability trial and all subjects have received the last dapiglutide dose for the safety and tolerability trial with key results expected later this year (ClinicalTrials.gov Identifier: NCT04612517). Obesity Amylin Later this year we expect to initiate a Phase 1 clinical trial with our long-acting Amylin analog ZP8396 that is being developed as a potential once-weekly treatment of obesity and type 2 diabetes. BI 456906: Long-acting GLP-1/GLU dual agonist for obesity and/or diabetes (with Boehringer Ingelheim) The GLP-1/glucagon dual agonist activates two key gut hormone receptors simultaneously and may offer better blood sugar and weight-loss control than current single-hormone receptor agonist treatments. The lead molecule BI 456906 is targeting treatment of obesity, diabetes, and non-alcoholic steatohepatitis (NASH). At Obesity Week in November this year Boehringer Ingelheim will present 16-week body weight data from the BI 456906 Phase 1b trial. Three parallel Phase 2 trials are ongoing. All subjects have been randomized in the first phase 2 trial which evaluates the dose-relationship of BI 456906 on HbA1c from baseline to 16 weeks relative to placebo in 410 people with diabetes (ClinicalTrials.gov Identifier: NCT04153929). Secondary objectives are to assess the effect on change in body weight and an open-label comparator (semaglutide) will allow for comparison of the effects against a pure GLP-1R agonist. The second Phase 2 randomized double-blind placebo-controlled dose-finding trial will evaluate BI 456906 in people with obesity or who are overweight with a BMI 27 kg/m2 or higher without diabetes (ClinicalTrials.gov Identifier: NCT04667377). Participants will receive a subcutaneous injection of either BI 456906 or placebo once a week for the duration of the trial. The primary endpoint of this trial is the percentage change in body weight at week 46 compared to placebo. The third Phase 2 randomized double-blind placebo-controlled dose-finding trial will evaluate BI 456906 in people with NASH and liver fibrosis (F2/F3) with and without diabetes (ClinicalTrials.gov Identifier: NCT04771273). The primary endpoint of this trial is the histological improvement of steatohepatitis without worsening of fibrosis after 48 weeks of treatment. Participants will receive a weekly subcutaneous injection of either different doses of BI 456906 or placebo for the duration of the trial. The program has received Fast Track designation from the U.S. FDA. Boehringer Ingelheim is funding all research, development and commercialization activities related to the treatment. Zealand Pharma is eligible to receive up to EUR 345 million in outstanding milestone payments, and high-single to low-double digit royalties on global sales. Inflammation Zealand Pharma is pursuing multiple pre-clinical programs in inflammatory diseases which will be detailed more as they progress through development. Complement inhibitors (with Alexion, AstraZeneca Rare Disease) Zealand Pharma and Alexion Pharmaceuticals announced in March 2019 that they will collaborate on the discovery and development of novel peptide therapies for complement-mediated diseases. Under the terms of the agreement, Alexion and Zealand Pharma entered into an exclusive collaboration for the discovery and development of subcutaneously delivered peptide therapies directed to up to four complement pathway targets. The lead program is a long-acting inhibitor of Complement C3 which has the potential to treat a broad range of complement mediated diseases. Zealand Pharma will lead the joint discovery and research efforts through the preclinical stage, and Alexion will lead development efforts beginning with IND filing and Phase 1 trials. We are looking to initiate a Phase 1 trial of the C3 inhibitor in 2022. For the lead target, Zealand Pharma is eligible to receive up to USD 610 million in development and sales milestone payments, plus royalties on global sales in the high single to low double digits. In addition, Alexion has the option to select up to three additional targets with Zealand Pharma eligible for USD 15 million upfront per target plus development/regulatory milestones for each target selected similar to the lead target with slightly reduced commercial milestones and royalties. Additional Updates On December 18, 2020 Amyndas Pharmaceuticals S.A. and Amyndas Pharmaceuticals LLC filed a complaint in the U.S. District Court for the District of Massachusetts, which named Alexion Pharmaceuticals, Inc., Zealand Pharma A/S and Zealand Pharma U.S., Inc. as defendants.  The complaint alleges claims against the Zealand Pharma A/S (and its U.S. subsidiary) and its collaboration partner Alexion Pharmaceuticals, Inc. (“Alexion”) related to Zealand Pharma A/S’s collaboration with Alexion on C3 peptide-based assets. The complaint alleges federal and state law claims, including claims for breach of confidentiality agreements, trade secret misappropriation and unfair competition.  The complaint seeks an unspecified amount of damages plus interest and injunctive relief. On June 8, 2021 the District Court dismissed the proceedings against Zealand Pharma U.S., Inc. for failure to state a claim, and dismissed the claims against Zealand Pharma A/S on the ground that the matter should be heard in the courts of Denmark.  On July 6, 2021 Amyndas moved the District Court to reconsider its dismissal of the claims against Zealand Pharma A/S (and its U.S. subsidiary).  Zealand Pharma A/S (and its U.S. subsidiary) have opposed Amyndas’s motion for reconsideration and the parties are currently awaiting a ruling on that motion. On 23 January 2020 Protagonist Therapeutics filed a demand for arbitration with the International Court of Arbitration of the International Chamber of Commerce (ICC) seeking a declaration that it has no past, present or future milestone or royalty payment obligations with respect to the compound it is advancing, PTG-300, alleging that the compound is not within the set of compounds to which such payment obligations apply. On August 4, 2021 the parties reached a mutually acceptable settlement of the proceedings and the agreement will continue between parties on agreed terms. Protagonist paid the sum of $2.5m as the first payment under the settlement agreement. Conference call today at 4 pm CEST / 10 am EDT Zealand Pharma’s management will host a conference call today at 4 pm CEST to present results through the first six months of 2021. Participating in the call will be Chief Executive Officer Emmanuel Dulac, Chief Financial Officer Matt Dallas, and Chief Medical and Development Officer Adam Steensberg. The presentation will be followed by a Q&A session with the presenters as well as the President of Zealand Pharma U.S., Frank Sanders. The conference call will be conducted in English, and the dial-in numbers are: A live audio webcast of the call, including an accompanying slide presentation, will be accessible from the Investor section of Zealand Pharma’s website. Participants are advised to register for the webcast approximately 10 minutes before the start. A recording of the event will be available on the Investor section of Zealand Pharma’s website following the call. Upcoming events Zealand Pharma plans to publish results for the third quarter of 2021 on November 11, 2021. Total number of shares and voting rights in Zealand Pharma as of June 30, 2021 Number of shares (nominal value of DKK 1 each): 43,541,838 which is an increase of 113,646 from 43,428,192 as of March 31, 2021. Therefore, the current Share capital is (nominal value in DKK): 43,541,838. Number of voting rights: 43,541,838 About Zealand Pharma A/S Zealand Pharma A/S (Nasdaq: ZEAL) ("Zealand") is a biotechnology company focused on the discovery, development, and commercialization of peptide-based medicines. More than 10 drug candidates invented by Zealand have advanced into clinical development, of which two have reached the market. Zealand’s robust pipeline of investigational medicines includes three candidates in late-stage development. Zealand markets V-Go®, a basal-bolus insulin delivery option for people with diabetes, and has received FDA approval for Zegalogue, (dasiglucagon), the first and only glucagon analogue for the treatment severe hypoglycemia in pediatric and adult patients with diabetes aged 6 and above. With this two products Zealand can commercialize its these two products using its own dedicated sales force and has established itself as a fully integrated biotechnology company. License collaborations with Boehringer Ingelheim and AstraZeneca create opportunity for more patients to potentially benefit from Zealand-invented peptide investigational agents currently in development. Zealand was founded in 1998 in Copenhagen, Denmark, and has presence throughout the U.S. that includes key locations in Boston, and Marlborough (MA). For more information about Zealand’s business and activities, please visit . Zegalogue® and V-Go® are registered trademarks of Zealand Pharma A/S. Safe Harbor / Forward-Looking Statements This press release contains “forward-looking statements”, as that term is defined in the Private Securities Litigation Reform Act of 1995, as amended, that provide Zealand Pharma’s expectations or forecasts of future events regarding the research, development and commercialization of pharmaceutical products and the company’s Financial Guidance for 2021.  These forward-looking statements may be identified by words such as “aim,” “anticipate,” “believe,” “could,” “estimate,” “expect,” “forecast,” “goal,” “intend,” “may,” “plan,” “possible,” “potential,” “will,” “would” and other words and terms of similar meaning. You should not place undue reliance on these statements, or the scientific data presented. The reader is cautioned not to rely on these forward-looking statements.  Such forward-looking statements are subject to risks, uncertainties and inaccurate assumptions, which may cause actual results to differ materially from expectations set forth herein and may cause any or all of such forward-looking statements to be incorrect, and which include, but are not limited to, unexpected costs or delays in clinical trials and other development activities due to adverse safety events or otherwise; unexpected concerns that may arise from additional data, analysis or results obtained during clinical trials; our ability to successfully market both new and existing products; changes in reimbursement rules and governmental laws and related interpretation thereof; government-mandated or market-driven price decreases for our products; introduction of competing products; production problems; unexpected growth in costs and expenses; our ability to integrate operate businesses in varying geographies; failure to protect and enforce our data, intellectual property and other proprietary rights and uncertainties relating to intellectual property claims and challenges; regulatory authorities may require additional information or further studies, or may reject, fail to approve or may delay approval of our drug candidates or expansion of product labeling; failure to obtain regulatory approvals in other jurisdictions; exposure to product liability and other claims; interest rate and currency exchange rate fluctuations; unexpected contract breaches or terminations; and the direct and indirect impacts of the ongoing COVID-19 pandemic on our business, results of operations and financial condition. If any or all of such forward-looking statements prove to be incorrect, our actual results could differ materially and adversely from those anticipated or implied by such statements. The foregoing sets forth many, but not all, of the factors that could cause actual results to differ from our expectations in any forward-looking statement.  All such forward-looking statements speak only as of the date of this press release and are based on information available to Zealand Pharma as of the date of this release. We do not undertake to update any of these forward-looking statements to reflect events or circumstances that occur after the date hereof.  Information concerning pharmaceuticals (including compounds under development) contained within this material is not intended as advertising or medical advice. NOTE: DKK/USD Exchange rates used: June 30, 2021 = 6.257 and June 30, 2020 = 6.6553 For further information, please contact: For U.S. Media Key figures * DKK thousand Notes: * The acquisition of the business from Valeritas is only reflected in key figures covering the period since April 2, 2020 being the acquisition date.(1) Zealand expects to be eligible to receive up to DKK 5.5 million in Danish corporate tax benefit related to R&D expenses incurred for 2021, of which DKK 2.8 million has been recognized for the period ended June 30, 2021.(2) Equity ratio is calculated as equity at the balance sheet date divided by total assets at the balance sheet date.(3) Free cash flow is calculated as the sum of cash flows from operating activities and purchase of property, plant and equipment.(4) Market capitalization is calculated as outstanding shares at the balance sheet date times the share price at the balance sheet date.(5) Equity per share is calculated as shareholders' equity divided by total number of shares less treasury shares. Financial review Comparative figures for the corresponding period in 2020 are shown in brackets except for the financial position, which expresses the comparative figures as of December 31, 2020. The condensed interim financial statements are prepared in accordance with IAS 34 Interim Financial Reporting, as issued by the International Accounting Standards Board (IASB) and as adopted by the EU, and additional requirements of the Danish Financial Statements Act. The interim condensed consolidated financial statements are presented in DKK, which is also the functional currency of the Company. Financial results Revenue Increase in revenue from the sale of goods is primarily attributable to the sales of V-Go in the period. Due to the Valeritas asset purchase agreement closing in April 2020, product revenue for V-Go in the H1 2020 is only for the last three months of the period. The decrease in license and milestone revenue is mainly due to a milestone payment of DKK 149.1 million triggered in June 2020 from our partnership agreement with Boehringer Ingelheim. Gross margin The decrease in gross margin is due to the milestone payment of DKK 149.1 million triggered in June 2020 from our partnership agreement with Boehringer Ingelheim. Research and development expenses The decrease in research and development expenses mainly relates to a reduction in costs for Zegalogue due to the March NDA approval. The reduction in costs related to Zegalogue is partially offset by costs incurred by our late-stage clinical programs for dasiglucagon and glepaglutide. Sales and marketing expenses The increase in sales and marketing expenses is related to efforts for the Zegalogue launch as well as continued commercial support for the V-Go wearable insulin deliver device. Due to the Valeritas asset purchase agreement closing in April 2020, sales and marketing expenses in the H1 2020 are only for the last three months of the period. Administrative expenses The increase in administrative expenses is a relating to costs related to the buildup and operations of the US subsidiary which supports the commercial infrastructure as well as general and administrative purposes. Substantial US operations were acquired in April 2020 following the close of the Valeritas asset purchase agreement. Operating result The operating result reflects gross margin, research and development expenses, sales and marketing and administrative expenses, as discussed above. Financial income and financial expenses Financial income and financial expenses, which we refer to collectively as net financial items, consist of interest income and expense, dividend, banking fees and impact from adjustments related to foreign exchange rates. Result before tax Result before tax reflects the operating result and net financial items, as discussed above. Income tax The net income tax income is mainly impacted by the tax deduction in Denmark, a prior period correction offset by the taxable income in US No deferred tax asset regarding the Danish parent company has been recognized in the statement of financial position due to uncertainty as to whether tax losses carried forward can be utilized within the near term. Net result The decrease in the net result is primarily a result of increased sales and marketing and administrative expenses. The expense increase is due to the 1H of 2020 only having commercial infrastructure effective April 2020 following the Valeritas asset purchase agreement. In addition, there was a one-time milestone of DKK 149.1 million triggered in June 2020 from our partnership agreement with Boehringer Ingelheim. Liquidity and capital resources Equity Equity ratio is calculated as equity at the balance sheet date divided by total assets at the balance sheet date. The increase in equity was mainly driven by a capital increase in January 2021 amounting to DKK 748.9 million offset by the loss for the period. Cash, cash equivalents and Marketable securities The increase was mainly driven by the capital increase in January 2021 amounting to DKK 748.9 million offset by cash spent in the period. Cash flow The increase in cash used in operating activities from the same period in 2020 is mainly related to our sales and marketing and administrative expenses increasing as a result of the commercial activities and support for Zegalogue and the V-Go wearable insulin delivery device. In the H1 of 2020 Zealand only had the sales and marketing expense and infrastructure effective April 2020 following the Valeritas asset purchase agreement. Cash used in investing activities in the first six months of 2021 related mainly to acquisition of tangible assets. Cash from financing activities increased primarily because of the January financing with an aggregate gross amount of DKK 748.9 million. Cash from financing activities for the six months ended June 30, 2020 was mainly related June financing of gross DKK 657.7 million but also a private placement of gross DKK 137.2 million. Risk factors This interim report contains forward-looking statements, including forecasts of future expenses as well as expected business-related events. Such statements are subject to risks and uncertainties as various factors, some of which are beyond the control of Zealand, may cause actual results and performance to differ materially from the forecasts made in this interim report. Without being exhaustive, such factors include e.g. the impact of the global COVID-19 pandemic, interest rate and currency exchange rate fluctuations, delay or failure of clinical trials and other development activities, production problems, unexpected contract breaches or terminations, government-mandated or market-driven price decreases for Zealand's products, introduction of competing products, Zealand's ability to successfully market both new and existing products, exposure to product liability and other lawsuits, changes in reimbursement rules and governmental laws and related interpretation thereof, unexpected growth in costs and expenses, and Zealand’s ability to integrate businesses in varying geographies with different commercial and operating characteristics. In particular, the global COVID-19 pandemic could potentially materially adversely impact our business and financial performance, including the timing of our clinical trials, projected regulatory approval timelines, our supply chain and sales of our approved products, as well as our Financial Guidance for 2021 in this interim report, particularly because the COVID-19 pandemic continues to evolve, and its breadth and significance on our business and financial performance is uncertain. A more extensive description of risk factors can be found in the 2020 Annual Report under the section Risk management and internal control. Management’s statement on the interim report The Board of Directors and the Management have considered and adopted the interim report of Zealand Pharma A/S for the three- and six-months periods ended June 30, 2021. The condensed consolidated interim financial statements are prepared in accordance with IAS 34 Interim Financial Reporting, as issued by the International Accounting Standards Board (IASB) and as adopted by the EU, and additional requirements of the Danish Financial Statements Act. In our opinion, the interim report gives a true and fair view of the Group’s assets, equity and liabilities and financial position at June 30, 2021 as well as of the results of the Group’s operations and cash flow for the period January 1 – June 30, 2021. Moreover, in our opinion, the Management’s Review gives a true and fair view of the development in the Company’s operations and financial conditions, of the net result for the periods and the financial position while also describing the most significant risks and uncertainty factors that may affect the Group. Copenhagen, August 12, 2021 Management Board of Directors Independent auditor’s report To the shareholders of Zealand Pharma A/S We have reviewed the interim condensed consolidated financial statements of Zealand Pharma A/S for the three- and six-month periods ended June 30, 2021, which comprise a condensed consolidated income statement and statement of comprehensive income for the three and six month periods ended June 30, 2021, statement of financial position at June 30, 2021, and statement of changes in equity and statement of cash flow, for the six month period ended June 30, 2021, and notes. The interim condensed consolidated financial statements are prepared in accordance with IAS 34 Interim Financial Reporting, as issued by the International Accounting Standards Board (IASB) and as adopted by the EU, and additional requirements of the Danish Financial Statements Act. Management's responsibilities for the interim condensed consolidated financial statementsManagement is responsible for the preparation of interim condensed consolidated financial statements in accordance with IAS 34 Interim Financial Reporting, as issued by the International Accounting Standards Board (IASB) and as adopted by the EU, and additional requirements of the Danish Financial Statements Act and for such internal control as Management determines is necessary to enable the preparation of interim condensed consolidated financial statements that are free from material misstatement, whether due to fraud or error. Auditor's responsibilitiesOur responsibility is to express a conclusion on the interim condensed consolidated financial statements. We conducted our review in accordance with the International Standard on Review of Interim Financial Information Performed by the Independent Auditor of the Entity and additional requirements applicable in Denmark. This requires us to conclude whether anything has come to our attention that causes us to believe that the interim condensed consolidated financial statements, taken as a whole, are not prepared, in all material respects, in accordance with IAS 34 Interim Financial Reporting, as issued by the International Accounting Standards Board (IASB) and as adopted by the EU, and additional requirements of the Danish Financial Statements Act. This standard also requires us to comply with ethical requirements. A review of the interim condensed consolidated financial statements in accordance with the International Standard on Review of Interim Financial Information Performed by the Independent Auditor of the Entity is a limited assurance engagement. The auditor performs procedures primarily consisting of making enquiries of Management and others within the company, as appropriate, applying analytical procedures and evaluate the evidence obtained. The procedures performed in a review are substantially less that those performed in an audit conducted in accordance with the International Standards on Auditing. Accordingly, we do not express an audit opinion on the interim condensed consolidated financial statements. Conclusion Based on our review, nothing has come to our attention that causes us to believe that these interim condensed consolidated financial statements are not prepared, in all material respects, in accordance with IAS 34 Interim Financial Reporting, as issued by the International Accounting Standards Board (IASB) and as adopted by the EU, and additional requirements of the Danish Financial Statements Act. Copenhagen, August 12, 2021 EY Godkendt Revisionspartnerselskab CVR no. 30 70 02 28 Interim condensed consolidated financial statements Interim condensed consolidated income statement for the three- and six-months periods ended June 30, 2021 and 2020. Interim condensed consolidated statement of comprehensive income (loss) for the three- and six-months periods ended June 30, 2021 and 2020. Interim condensed consolidated statements of cash flow for the six months periods ended June 30, 2021 and 2020 and for the twelve months period ended December 31, 2020 Interim condensed consolidated statements of financial position as of June 30, 2021 and December 31, 2020 Interim condensed consolidated statements of changes in equity for the six months periods ended June 30, 2021 and 2020 Note 1 - Basis of preparation and changes to the Group’s accounting policies Basis of preparation The interim condensed consolidated financial statements of Zealand Pharma A/S (“the Company”) have been prepared in accordance with IAS 34, Interim Financial Reporting, as issued by the International Accounting Standards Board (IASB) and as adopted by EU and additional requirements of the Danish Financial Statements Act. The interim condensed consolidated financial statements are presented in Danish kroner (DKK) which is also the functional currency of the parent company. The accounting policies used in the interim condensed consolidated financial statements are consistent with those used in the Company’s annual financial statement for the year ended December 31, 2020. New standards, interpretations and amendments adopted by the Group A few amendments apply for the first time in 2021, but do not have an impact on the interim condensed consolidated financial statements of the Group. Significant judgements and estimates In the preparation of the interim condensed consolidated financial statements, the Company’s management (“Management”) makes several accounting estimates that form the basis for the presentation, recognition and measurement of the Company’s assets and liabilities. In the application of the Company’s accounting policies, Management is required to make judgments, estimates and assumptions about the carrying amounts of assets and liabilities that are not readily apparent from other sources. The estimates and associated assumptions are based on historical experience and other factors that are considered to be relevant. Actual results may differ from these estimates. The estimates and underlying assumptions are reviewed on an ongoing basis. Revisions to accounting estimates are recognized in the period in which the estimate is revised if the revision affects only that period, or in the period of the revision and future periods if the revision affects both current and future periods. The estimates used are based on assumptions assessed as reasonable by Management; however, estimates are inherently uncertain and unpredictable. The assumptions can be incomplete or inaccurate, and unexpected events or circumstances might occur. Furthermore, the Company is subject to risks and uncertainties that might result in deviations in actual results compared with estimates. For further information regarding significant accounting estimates and judgments see note 1 in the Annual Report for 2020. Information on COVID-19 Our business, operations and clinical studies were, of course, impacted by the effects of COVID-19. Although our clinical studies continued without interruption during 2021 and 2020, there were delays and increased total costs arising from the implications of COVID-19. However, we have not recognized any write-offs, impairments of assets, or losses to onerous contracts due to COVID-19. The COVID-19 pandemic is also having an effect on other aspects of our business, including: our third-party manufacturers, and other third parties; albeit with no material effect or impact. The COVID-19 pandemic may, in the long-term, affect the productivity of our staff; our ability to attract, integrate, manage and retain qualified personnel or key employees; our global supply chains and relationships with vendors and other parties; significant disruption of global financial markets; and reduced ability to secure additional funding. We continuously monitor the COVID-19 pandemic and its potential impact on our business and financials. Note 2 - Revenue Revenue can be specified as follows: License revenue for the first six months of 2021 of DKK 10.3 million relate to the research and development agreement with Alexion Pharmaceuticals entered into in March 2019. Under the agreement DKK 87.4 million is accounted for as deferred revenue as of June 30, 2021. Milestone revenue for the first six months of 2021 of DKK 30.7 million relate to the Sanofi agreement. Zealand is still eligible for a payment from Sanofi of up to USD 10.0 million which is expected in 2022. Sale of goods revenue for the first six months of 2021 of DKK 91.1 million related to V-Go and Zegalogue. The net sales comprise of gross sales of DKK 175.8 million and discounts and rebates of DKK -84.7 million. Zealand is managed and operated as one business unit, which is reflected in the organizational structure and internal reporting. No separate lines of business or separate business entities have been identified with respect to any of the product candidates or geographical markets and no segment information is currently included in the internal reporting. Net sales in US for the six months period ended June 30, 2021 comprise DKK 101.5 million including license revenues and sale of goods. Net sales in Germany amounts to DKK 30.7 million including license and milestone revenue. No other country accounts for more than 10% of the net total sales. Note 3 – Royalty expense Royalty expense relates to cost to 3rd parties in connection with the Sanofi royalty agreement. Note 4 – Administrative, sale and marketing expenses The increase in administrative, sale and marketing expenses in H1 2021 over H1 2020 is due to the acquisition of Valeritas in April 2020. Note 5 – Financial income Recognized financial income can be specified as follows: Due to the development of the FX rates in Q2, the Company recognized a net currency loss amounting to DKK 11.1 million in the three-month period ended June 30, 2021. As a result, financial income and financial expense for the six-month period have been impacted by a net decrease of DKK 11.1 million. Note 6 - Financial expenses Recognized financial expenses can be specified as follows: Note 7 – Tax The tax amount recognized includes tax income of DKK 2.8 million relating to corporate tax benefit in Denmark, a correction to prior periods of DKK 7.3 million offset by tax expenses in US of DKK 2.6 million. Note 8 - Earnings/Loss per share The earnings/loss and weighted average number of ordinary shares used in the calculation of basic and diluted earnings/loss per share are as follows: For further information on the Employee incentive programs please see note 6 in the Annual Report for 2020. Note 9 – Intangible assets Intangible assets of DKK 55.3 million recognized as at June 30, 2021 as compared to DKK 57.5 million as of December 31, 2020. The decrease is primarily due to currency translation and amortization. The majority of the intangible assets occurred from the business combination of Valeritas activities on April 2, 2020. Since the 12 months period has expired all amounts are assessed to be final and no further adjustments relating to the business combination can occur. Note 10 – Property, plant and equipment The increase from DKK 85.0 million at December 31, 2020 to DKK 85.4 million as at June 30, 2021 is primarily related new assets under construction in Denmark offset by depreciation. Note 11 - Right of use assets and lease liabilities Right-of-use-assets of DKK 140.1 million and lease liability of DKK 143.8 million were recognized as at June 30, 2021 as compared to DKK 128.0 million and DKK 130.1 million, respectively, as of December 31, 2020. The increase is primarily related to currency translation and new smaller additions, offset by depreciations and payments. Note 12 - Financial instruments As of June 30, 2021 and December 31, 2020, the following financial instruments are measured at fair value through profit or loss: The fair value of marketable securities and other investments is based on Level 1 and Level 3, respectively, in the fair value hierarchy. No financial assets are based on Level 2. Other investments consist of a USD 5.4 million (December 31, 2020: USD 5.4 million) investment in Beta Bionics, Inc., the developer of iLet™, a fully integrated dual-hormone pump (bionic pancreas) for autonomous diabetes care. In determining fair value, Zealand considered the impact of any recent share capital issuances by Beta Bionics as an indicator of the fair value of the shares. In particular, Beta Bionics undertook a capital offering in June 2019 and subsequent infliction points was used as the basis for determining fair value. Measurement is considered a level 3 measurement. A net fair value adjustment of DKK 1.1 million from marketable securities and other investments have been recognized in financial expenses, as of June 30, 2021 (June 30, 2020: DKK 0.8 million in financial income). There were no transfers between levels 1, 2 and 3 for recurring fair value measurements during the interim periods ended June 30, 2021 and 2020, respectively. Note 13 - Inventories In 2021 a reversal of previous periods estimated write-down on prelaunch inventory with a net positive income statement effect of DKK 25.1 million on research and development expenses was recognized, as a consequence of our FDA approval of Zegalogue. Note 14 – Trade receivables The increase in Trade receivables from DKK 46.5 million at December 31, 2020 to DKK 99.3 million at June 30, 2021 is mainly related to extended payment terms with our larger customers and the DKK 30 million Sanofi milestone. Note 15 – Prepaid expenses The increase in Prepaid expenses from DKK 48.3 million at December 31, 2020 to DKK 51.1 million at June 30, 2021 is mainly relating to timing differences. Note 16 - Other receivables Other receivables are mainly related to VAT receivables in Denmark and Sweden. Note 17 - Cash and cash equivalents Note 18 - Changes in share capital The following changes have occurred in the share capital during the respective year-to-date interim periods: Note 19 – Treasury shares As part of the capital increase made in January 2021, a total of 200,000 shares were purchased through a share s. As the payment is not due yet, a liability of DKK 41.6 million is recognized under other liabilities. Refer to note 21. Further in June 2021, the Company started a share buyback program with an expected purchase of 154,187 shares or for an amount up to DKK 32,070,896. As of June 30, 2021, 20,000 shares were acquired under this program. The total number of treasury shares as of June 30, 2021 is 284,223 and will be used for long term incentive compensation plans. Note 20 – Trade payables The increase in Trade payables from DKK 70.4 million as of December 31, 2020 to DKK 76.2 million at June 30, 2021 is mainly relating to timing differences. Note 21 - Other liabilities The increase in other liabilities is mainly related to a one-off liability regarding purchase of treasury shares of DKK 45.3 million. This is offset by a decrease in Employee benefits due to the payout of bonus in Q1 2021 and lower CRO liabilities.Other payables comprises of payables to authorities etc. Note 22 - Contingent assets, liabilities and contractual obligations Contingent assets As of June 30, 2021, Zealand is still eligible for a payment from Sanofi of up to USD 10.0 million which is expected in 2022. However, it is Management’s opinion that the amount of any payment cannot be determined on a sufficiently reliable basis, and therefore the company has not recognized an asset in the statement of financial position of the Group. Contingent liabilities and contractual obligations As of June 30, 2021, total contractual obligations related to agreements with CROs amounted to DKK 347.1 million (DKK 107.9 million for 2021 and DKK 239.2 million for the years 2022 up to and including 2025). Zealand may be required to pay future development, regulatory and commercial milestones related to the acquisition of Encycle Therapeutics. Refer to note 13 in the 2020 Annual Report. Note 23 - Significant events after the reporting period None