Article
Author: De Moreuil, Claire ; Bini, Paola ; Dernis, Emanuelle ; Giannini, Margherita ; Codullo, Veronica ; Galempoix, Jean-Marc ; Hervier, Baptiste ; Meyer, Alain ; Cavagna, Lorenzo ; Dubost, Jean Jacques ; Ziane, Rahima ; Mariampillai, Kuberaka ; Séverac, François ; Bouchard-Marmen, Maude ; Bernardi, Livio ; Soulages, Antoine ; Sibilia, Jean ; Diamanti, Luca ; Piot, Jean-Maxime ; Duval, Fanny ; Poursac, Nicolas ; Arnaud, Laurent ; Mosca, Marta ; Barsotti, Simone ; Troyanov, Yves ; Sellam, Jérémie ; Couderc, Marion ; Geny, Bernard ; Sole, Guilhem ; Guichard, Isabelle ; Groza, Monica ; Lebrun, Delphine ; Prieto-González, Sergio ; Viallard, Jean-François ; Nannini, Carlotta ; Selva-O'Callaghan, Albert ; Terrier, Benjamin ; Blagojevic, Jelena ; Vernier, Nathalie ; Bellando-Randone, Silvia ; Tournadre, Anne ; Iannone, Florenzo ; Iaccarino, Luca ; Gottenberg, Jacques-Eric ; Vesperini, Veronique ; Guilpain, Philippe ; Marchioni, Enrico ; Maurier, Francois ; Pijnenburg, Luc ; Guffroy, Aurelien ; Hudson, Marie ; Emmi, Giacomo ; Rouanet, Eglantine ; Nadaj-Pakleza, Aleksandra
BACKGROUNDSome myopathies can lead to dropped head or bent spine syndrome (DH/BS). The significance of this symptom has not been studied in inflammatory myopathies (IM).OBJECTIVESTo assess the significance of DH/BS in patients with IM.METHODSPractitioners from five IM networks were invited to report patients with IM suffering from DH/BS (without other known cause than IM). IM patients without DH/BS, randomly selected in each participating centre, were included as controls at a ratio of 2 to 1.RESULTS49 DH/BS-IM patients (DH: 57.1%, BS: 42.9%) were compared with 98 control-IM patients. DH/BS-IM patients were older (65 years vs 53 years, p<0.0001) and the diagnosis of IM was delayed (6 months vs 3 months, p=0.009). Weakness prevailing in the upper limbs (42.9% vs 15.3%), dysphagia (57.1% vs 25.5%), muscle atrophy (65.3% vs 34.7%), weight loss (61.2% vs 23.5%) and loss of the ability to walk (24.5% vs 5.1%) were hallmarks of DH/BS-IM (p≤0.0005), for which the patients more frequently received intravenous immunoglobulins (65.3% vs 34.7%, p=0.0004). Moreover, DH/BS-IM patients frequently featured signs and/or complications of systemic sclerosis (SSc), fulfilling the American College of Rheumatology/European Alliance of Associations for Rheumatology criteria for this disease in 40.8% of the cases (vs 5.1%, p<0.0001). Distribution of the myopathy, its severity and its association with SSc were independently associated with DH/BS (p<0.05). Mortality was higher in the DH/BS-IM patients and loss of walking ability was independently associated with survival (p<0.05).CONCLUSIONIn IM patients, DH/BS is a marker of severity and is associated with SSc (scleromyositis).
