Churg-Strauss Syndrome

Results from the trial indicate that almost 60% of patients treated with Fasenra achieved remission. Credit: Mr. Ashi. Sae Yang/Shutterstock. The European Commission (EC) has approved AstraZeneca ‘s Fasenra (benralizumab) as an add-on therapy for adults with relapsing or refractory eosinophilic granulomatosis with polyangiitis (EGPA), a condition previously known as Churg-Strauss syndrome. Fasenra is the second biologic approved for the condition. The decision follows a positive opinion from the Committee for Medicinal Products for Human Use (CHMP) and is supported by results from the MANDARA Phase III study. EGPA is an uncommon, immune-mediated vasculitis that results from inflammation of small to medium-sized blood vessels, potentially causing multi-organ damage. It can be perilous if left untreated. Almost 50% of patients with EGPA also suffer from severe eosinophilic asthma and often experience sinus and nasal symptoms. See Also: Novartis’ Scemblix gains FDA approval for leukaemia GSK to acquire Chimagen’s CMG1A46 for autoimmune disease AstraZeneca bioPharmaceuticals business unit executive vice-president Ruud Dobber stated: “Today’s approval of Fasenra, with its convenient, single-monthly injection, is a positive step forward for patients with EGPA. “Fasenra has been a well-established treatment for many years in thousands of people with severe eosinophilic asthma and we are pleased to now offer a much-needed treatment option for those living with EGPA in Europe.” The randomised, double-blinded, active-controlled MANDARA study evaluated Fasenra’s efficacy and safety against mepolizumab in adult subjects with relapsing or refractory EGPA. It marked the first head-to-head non-inferiority trial of biologics in this patient group. The trial enrolled 140 patients, randomised into a 1:1 ratio, who received either a single 30mg subcutaneous injection of Fasenra or three distinct 100mg injections of mepolizumab subcutaneously every four weeks. Almost 60% of patients treated with Fasenra achieved remission, a rate comparable to those treated with mepolizumab. 41% of patients treated with Fasenra completely narrowed down oral corticosteroids (OCS). The safety and tolerability profile in the MANDARA trial was consistent with the medicine’s known profile. Developed by AstraZeneca and in-licensed from BioWa, a subsidiary of Kyowa Kirin , Fasenra is approved in more than 80 countries and regions, including Japan, the US, the EU and China.

New indication supported by the MANDARA trial, which showed nearly 60% of patients achieved remission and 41% of patients fully stopped taking oral corticosteroids LONDON, UK I October 28, 2024 I AstraZeneca’s Fasenra (benralizumab) has been approved in the European Union (EU) as an add-on treatment for adult patients with relapsing or refractory eosinophilic granulomatosis with polyangiitis (EGPA). EGPA is a rare, immune-mediated vasculitis that can result in damage to multiple organs, and without treatment, can be fatal. 1,2 The approval by the European Commission follows the positive opinion of the Committee for Medicinal Products for Human Use and was based on positive results from the MANDARA Phase III trial, published in The New England Journal of Medicine . 3 MANDARA was the first head-to-head non-inferiority trial of biologics in patients with EGPA. 4-6 Patients were randomised to receive either a single 30 mg subcutaneous injection of Fasenra , or three separate 100 mg subcutaneous injections of mepolizumab every four weeks. 3,4 In the trial, nearly 60% of Fasenra- treated patients achieved remission, which was comparable to mepolizumab-treated patients. 3 Data also showed 41% of Fasenra- treated patients fully tapered off oral corticosteroids (OCS) (vs. 26% in the comparator arm (difference: 16%; 95% CI: 1,31)). 3 Bernhard Hellmich, Chair of the Department of Internal Medicine, Rheumatology, and Immunology at the Medius Klinik Kirchheim, Teaching Hospital of the University of Tübingen in Germany, Co-Director of the Vasculitis Center Tübingen-Kirchhei, and MANDARA Principal Investigator said: “People living with EGPA suffer debilitating symptoms, organ damage and even death. Today’s approval provides an important treatment option for people living with EGPA in the EU. By directly targeting and removing eosinophilic inflammation with benralizumab, I hope that we will see more patients achieve remission as well as a reduction in the reliance on oral corticosteroids, which can cause serious and long-term side effects.” Ruud Dobber, Executive Vice President, BioPharmaceuticals Business Unit, AstraZeneca said: “We remain committed to helping patients with some of the hardest-to-treat diseases. Today’s approval of Fasenra, with its convenient, single-monthly injection is a positive step forward for patients with EGPA. Fasenra has been a well-established treatment for many years in thousands of people with severe eosinophilic asthma and we are pleased to now offer a much-needed treatment option for those living with EGPA in Europe.” The safety and tolerability profile for Fasenra in the MANDARA trial was consistent with the known profile of the medicine. 3 Approximately half of patients with EGPA have adult-onset severe eosinophilic asthma (SEA) and often have sinus and nasal symptoms. 2,7,8 Fasenra is only the second biologic approved to treat this disease. 5 Fasenra is currently approved as an add-on maintenance treatment for SEA in more than 80 countries, including the US, Japan, EU and China. 9-12 It is also approved in children and adolescents ages 6 and above in the US and Japan. Fasenra was approved for EGPA in the US in September. 13 Notes Eosinophilic granulomatosis with polyangiitis EGPA, formerly known as Churg-Strauss Syndrome, is a rare, immune-mediated inflammatory disease that is caused by inflammation of small to medium-sized blood vessels. 1,2 It is estimated that 118,000 people throughout the world live with EGPA. 14 EGPA can result in damage to multiple organs, including lungs, upper airway, skin, heart, gastrointestinal tract and nerves. 2 The most common symptoms and signs include extreme fatigue, weight loss, muscle and joint pain, rashes, nerve pain, sinus and nasal symptoms, and shortness of breath. 2,15 Without treatment, the disease may be fatal. 2 Almost half (47%) of patients do not achieve remission with current treatments. 15 There are limited treatment options for EGPA. Patients are often treated with chronic high-dose OCS and experience recurrent relapses when attempting to taper off OCS. 16,17 MANDARA MANDARA was a Phase III, randomised, double-blinded, active-controlled trial, which compared the efficacy and safety of Fasenra to mepolizumab in adult patients with relapsing or refractory EGPA. 4 In the trial, 140 patients were randomised 1:1 to receive either a single 30mg subcutaneous injection of Fasenra or three separate 100mg subcutaneous injections of the active comparator every four weeks. 3 The primary endpoint was the proportion of patients who were in remission at both weeks 36 and 48. 4 Remission is defined as Birmingham Vasculitis Activity Score (BVAS)=0 and OCS dose less than or equal to 4 mg/day. 4 A secondary endpoint was the proportion of patients who were able to fully taper off OCS at weeks 48 through 52. 3 The primary statistical analysis was to demonstrate non-inferiority of Fasenra versus mepolizumab based on the primary endpoint. 3 Fasenra Fasenra (benralizumab) is currently approved in more than 80 countries, including the US, Japan, EU and China. 9-12 Fasenra has been prescribed to over 130,000 patients globally. 18 Fasenra is in development for other diseases including chronic obstructive pulmonary disease and hypereosinophilic syndrome. 19,20 Fasenra was developed by AstraZeneca and is in-licensed from BioWa, Inc., a wholly owned subsidiary of Kyowa Kirin Co., Ltd., Japan. AstraZeneca in Respiratory & Immunology Respiratory & Immunology, part of AstraZeneca BioPharmaceuticals, is a key disease area and growth driver to the Company. AstraZeneca is an established leader in respiratory care with a 50-year heritage and a growing portfolio of medicines in immune-mediated diseases. The Company is committed to addressing the vast unmet needs of these chronic, often debilitating, diseases with a pipeline and portfolio of inhaled medicines, biologics and new modalities aimed at previously unreachable biologic targets. Our ambition is to deliver life-changing medicines that help eliminate COPD as a leading cause of death, eliminate asthma attacks and achieve clinical remission in immune-mediated diseases. AstraZeneca AstraZeneca (LSE/STO/Nasdaq: AZN) is a global, science-led biopharmaceutical company that focuses on the discovery, development, and commercialisation of prescription medicines in Oncology, Rare Diseases, and BioPharmaceuticals, including Cardiovascular, Renal & Metabolism, and Respiratory & Immunology. Based in Cambridge, UK, AstraZeneca’s innovative medicines are sold in more than 125 countries and used by millions of patients worldwide. Please visit astrazeneca.com and follow the Company on social media @AstraZeneca References 1. Furuta S, et al . Update on eosinophilic granulomatosis with polyangiitis. Allergol Int. 2019;68:430-436. 2. American Partnership for Eosinophilic Disorders. Eosinophilic Granulomatosis with Polyangiitis (EGPA). Available at: https://apfed.org/about-ead/eosinophilic-granulomatosis-with-polyangiitis/ . [Last accessed: October 2024]. 3. Wechsler ME, et al . Benralizumab versus Mepolizumab for Eosinophilic Granulomatosis with Polyangiitis. N Engl J Med . 2024;390(10):911-921. 4. Clinicaltrials.gov. Efficacy and Safety of Benralizumab in EGPA Compared to Mepolizumab. (MANDARA). Available at: https://classic.clinicaltrials.gov/ct2/show/NCT04157348 . [Last accessed: October 2024]. 5. Mepolizumab US prescribing information. Available from: https://www.fda.gov/files/drugs/published/125526-Mepolizumab-Clinical-PREA.pdf [Last accessed: October 2024]. 6. AstraZeneca plc. MANDARA Phase III data published in New England Journal of Medicine show remission is an achievable goal in eosinophilic granulomatosis with polyangiitis (EGPA) with Fasenra. Available at: https://www.astrazeneca.com/media-centre/medical-releases/mandara-phase-iii-data-published-new-england-journal-medicine-show-remission-achievable-goal-eosinophilic-granulomatosis-polyangiitis-egpa-fasenra.html . [Last accessed: October 2024]. 7. Cottin V, et al . Respiratory manifestations of eosinophilic granulomatosis with polyangiitis (Churg–Strauss). Eur Respir J . 2016;48:1429-1441. 8. Heaney L et al . Eosinophilic and Noneosinophilic Asthma: An Expert Consensus Framework to Characterize Phenotypes in a Global Real-Life Severe Asthma Cohort. Chest . 2021 Sep;160(3):814-830. 9. AstraZeneca news release. Available at: https://www.astrazeneca.com/media-centre/press-releases/2019/fasenra-approved-in-the-us-for-self-administration-in-a-new-pre-filled-auto-injector-the-fasenra-pen-04102019.html# . [Last accessed: October 2024]. 10. AstraZeneca news release. Available at: https://www.astrazeneca.com/media-centre/press-releases/2019/fasenra-receives-positive-eu-chmp-opinion-for-self-administration-and-the-new-fasenra-pen-a-pre-filled-single-use-auto-injector-01072019.html# . [Last accessed: October 2024]. 11. AstraZeneca Annual Report 2023. Available at: https://www.astrazeneca.com/content/dam/az/Investor_Relations/annual-report-2023/pdf/AstraZeneca_AR_2023.pdf . [Last accessed: October 2024]. 12. AstraZeneca news release. Available at: https:// www.astrazeneca.com/media-centre/press-releases/2024/fasenra-approved-in-china-for-the-treatment-of-severe-eosinophilic-asthma.html . [Last accessed: October 2024]. 13. Fasenra (benralizumab) US prescribing information; September 2024. Available at: https://www.accessdata.fda.gov/drugsatfda_docs/label/2024/761070s021lbl.pdf [Last accessed: October 2024]. 14. AstraZeneca Data on file. 2024. REF- 244520. 15. Wechsler ME, et al . Mepolizumab or Placebo for Eosinophilic Granulomatosis with Polyangiitis. N Engl J Med . 2017:376;1921-1932. 16. Baldini C, et al. Clinical Manifestations and Treatment of Churg-Strauss Syndrome. Rheum Dis Clin N Am . 2010;36:527–543. 17. Bell CF, et al . Burden of illness and costs associated with eosinophilic granulomatosis with polyangiitis: evidence from a managed care database in the United States. J Manag Care Spec Pharm . 2021;27(9):1249-1259. 18. AstraZeneca data on file. 2024. REF-235794. 19. Clinicaltrials.gov. Efficacy and Safety of Benralizumab in Moderate to Very Severe Chronic Obstructive Pulmonary Disease (COPD) With a History of Frequent Exacerbations (RESOLUTE). Available from: https://clinicaltrials.gov/ct2/show/NCT04053634 [Last accessed: October 2024]. 20. Clinicaltrials.gov. A Phase 3 Study to Evaluate the Efficacy and Safety of Benralizumab in Patients With Hypereosinophilic Syndrome (HES) (NATRON). Available from: https://clinicaltrials.gov/ct2/show/NCT04191304 [Last accessed: October 2024]. SOURCE: Fasenra

JAK inhibitors are a class of medications that work by blocking Janus kinases (JAKs), enzymes that are involved in the signaling pathways of various cytokines and growth factors. JAK inhibitors offer a more targeted approach to treating immune-mediated diseases compared to traditional therapies like corticosteroids or conventional immunosuppressants; this specificity reduces side effects and increases efficacy and is a major driver for the demand for JAK inhibitors. LAS VEGAS, Oct. 22, 2024 /PRNewswire/ -- DelveInsight's ' JAK Inhibitors Pipeline Insight 2024 ' report provides comprehensive global coverage of pipeline JAK inhibitors in various stages of clinical development, major pharmaceutical companies are working to advance the pipeline space and future growth potential of the JAK inhibitors pipeline domain. Key Takeaways from the JAK Inhibitors Pipeline Report DelveInsight's JAK inhibitors pipeline report depicts a robust space with 50+ active players working to develop 55+ pipeline JAK inhibitors. Key JAK inhibitors companies such as Incyte Corporation, Celon Pharma, Aclaris Therapeutics, Sareum, AstraZeneca, Incyte Corporation, Ajax Therapeutics, Pfizer, GSK, Dizal Pharmaceutical, Confluence Life Sciences, Celon Pharma, Incyte Corporation, Arcutis Biotherapeutics/Reistone Biopharma, Esker Therapeutics, Bristol-Myers Squibb, Chia Tai Tianqing Pharmaceutical and others are evaluating new JAK Inhibitors drugs to improve the treatment landscape. Promising pipeline JAK inhibitors such as Povorcitinib, CPL409116, ATI-2138, SDC 1802, AZD 4604, INCB-160058, Research programme: JAK2 inhibitors, Ritlecitinib, Momelotinib, DZD4205, ATI 2138, CPL 409116, Itacitinib, Ivarmacitinib, ESK 001, Repotrectinib, Rovadicitinib, and others are under different phases of JAK inhibitors clinical trials. In October 2024, Pelabresib in combination with ruxolitinib showed benefit in treating patients with JAK Inhibitor-Naïve Myelofibrosis significantly reduced splenomegaly, and improved anemia that has not been previously treated with Janus kinase inhibitors (JAKis). In September 2024, Eli Lilly and Company and EVA Pharma announced that the companies have agreed to expand access to baricitinib to an estimated 20,000 people in 49 low- to middle-income countries in Africa by 2030. In May 2024, Ajax Therapeutics, Inc., announced that it had received clearance for its Investigational New Drug application from the U.S. Food and Drug Administration (FDA) to initiate a Phase I clinical study of AJ1–11095, a first-in-class Type II JAK2 inhibitor, for the treatment of patients with myelofibrosis. In February 2024, Novartis announced that it intends to acquire MorphoSys. The acquisition, which was unanimously approved by the boards of both Novartis and MorphoSys, is expected to close in the first half of 2024, subject to customary closing conditions. In January 2024, NS Pharma received orphan drug designation (ODD) from the European Commission (EC) for NS-229, which is being developed to treat eosinophilic granulomatosis with polyangiitis (EGPA), a rare autoimmune disease. In December 2023, Sun Pharmaceuticals Inc. announced that it has entered a licensing agreement with Aclaris Therapeutics Inc., the company announced in a regulatory filing. Under the agreement, Aclaris granted Sun Pharma exclusive rights under certain patents for the use of deuruxolitinib, Sun Pharma's JAK inhibitor, or other isotopic forms of ruxolitinib, to treat alopecia areata (AA) or androgenetic alopecia (AGA). Request a sample and discover the recent advances in JAK inhibitors drugs @ JAK Inhibitors Pipeline Report The JAK inhibitors pipeline report provides detailed profiles of pipeline assets, a comparative analysis of clinical and non-clinical stage JAK inhibitors drugs, inactive and dormant assets, a comprehensive assessment of driving and restraining factors, and an assessment of opportunities and risks in the JAK inhibitors clinical trial landscape. JAK Inhibitors Overview Janus kinase (JAK) inhibitors are a class of medications designed to interfere with the activity of the Janus kinase family of enzymes, which play a crucial role in the signaling pathways of immune responses. These enzymes are key mediators in transmitting signals from cytokines and growth factors, which regulate blood cell production and immune system function. By blocking these signals, JAK inhibitors help to control the overactive immune response seen in several inflammatory and autoimmune diseases. They have shown significant efficacy in treating conditions like rheumatoid arthritis, psoriatic arthritis, and ulcerative colitis, among others. The most common JAK inhibitors include tofacitinib, baricitinib, and upadacitinib, which are administered orally. Beyond their role in autoimmune diseases, JAK inhibitors are also being explored for use in treating certain cancers and myeloproliferative disorders, where abnormal JAK signaling contributes to uncontrolled cell growth. In diseases like myelofibrosis and polycythemia vera, JAK inhibitors can help reduce symptoms and improve quality of life. However, there are potential side effects associated with their use, including increased susceptibility to infections, blood clots, and cardiovascular risks, given their impact on the immune system. As research continues, the therapeutic potential of JAK inhibitors is expanding, offering new hope for patients with difficult-to-treat conditions. Find out more about JAK inhibitors drugs @ JAK Inhibitors Analysis A snapshot of the Pipeline JAK Inhibitors Drugs mentioned in the report: Learn more about the emerging JAK inhibitors @ JAK Inhibitors Clinical Trials JAK Inhibitors Therapeutics Assessment The JAK inhibitors pipeline report proffers an integral view of the emerging JAK inhibitors segmented by stage, product type, molecule type, and route of administration. Scope of the JAK Inhibitors Pipeline Report Coverage: Global Therapeutic Assessment By Product Type: Mono, Combination, Mono/Combination Therapeutic Assessment By Clinical Stages: Discovery, Pre-clinical, Phase I, Phase II, Phase III Therapeutics Assessment By Route of Administration: Oral, Intravenous, Subcutaneous, Parenteral, Topical Therapeutics Assessment By Molecule Type: Recombinant fusion proteins, Small molecule, Monoclonal antibody, Peptide, Polymer, Gene therapy Key JAK Inhibitors Companies: Incyte Corporation, Celon Pharma, Aclaris Therapeutics, Sareum, AstraZeneca, Incyte Corporation, Ajax Therapeutics, Pfizer, GSK, Dizal Pharmaceutical, Confluence Life Sciences, Celon Pharma, Incyte Corporation, Arcutis Biotherapeutics/Reistone Biopharma, Esker Therapeutics, Bristol-Myers Squibb, Chia Tai Tianqing Pharmaceutical, and others. Key JAK Inhibitors Pipeline Therapies: Povorcitinib, CPL409116, ATI-2138, SDC 1802, AZD 4604, INCB-160058, Research programme: JAK2 inhibitors, Ritlecitinib, Momelotinib, DZD4205, ATI 2138, CPL 409116, Itacitinib, Ivarmacitinib, ESK 001, Repotrectinib, Rovadicitinib, and others. Dive deep into rich insights for new JAK inhibitors, visit @ JAK Inhibitors Drugs Table of Contents For further information on the JAK inhibitors pipeline therapeutics, reach out @ JAK Inhibitors Therapeutics Related Reports JAK Inhibitors Market JAK Inhibitors Market Size, Target Population, Competitive Landscape & Market Forecast – 2034 report provides comprehensive insights about the pipeline landscape, pipeline drug profiles, including clinical and non-clinical stage products, and the key JAK inhibitors companies, including Eli Lilly and Company, Suzhou Zelgen Biopharmaceuticals Co., Ltd, Incyte Corporation, Kartos Therapeutics, Inc., Dompé Farmaceutici S.p.A, Sanofi, Kiniksa Pharmaceuticals, Ltd., Celgene, Abivax S.A., Telios Pharma, Inc., AstraZeneca, Karyopharm Therapeutics Inc, TWi Biotechnology, Inc., Geron Corporation, Ajax Therapeutics, Inc., among others. 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