In inflammatory myopathies, dropped head/bent spine syndrome is associated with scleromyositis: an international case–control study
Luc Pijnenburg, Margherita Giannini, Maude Bouchard-Marmen, Laurent Arnaud, Simone Barsotti, Silvia Bellando-Randone, L. Bernardi, Paola Bini, Jelena Blagojević, Veronica Codullo, Marion Couderc, Claire de Moreuil, E. Dernis, Luca Diamanti, Jean‐Jacques Dubost, Fanny Duval, Giacomo Emmi, Jean‐Marc Galempoix, Bernard Gény, Jacques‐Eric Gottenberg, M. Groza, Aurélien Guffroy, Isabelle Guichard, Philippe Guilpain, B. Hervier, Marie Hudson, Luca Iaccarino, Florenzo Iannone, Delphine Lebrun, Enrico Marchioni, K. Mariampillai, F. Maurier, Marta Mosca, Aleksandra Nadaj‐Pakleza, Carlotta Nannini, Jean-Maxime Piot, Sergio Prieto‐González, Nicolas Poursac, Eglantine Rouanet, Jérémie Sellam, Albert Selva-O’Callaghan, François Séverac, Jean Sibilia, Guilhem Solé, Antoine Soulages, Benjamin Terrier, Anne Tournadre, Yves Troyanov, N. Vernier, Véronique Vesperini, Jean‐François Viallard, Rahima Ziane, Lorenzo Cavagna, Alain Meyer
Abstract
BACKGROUND: Some 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). OBJECTIVES: To assess the significance of DH/BS in patients with IM. METHODS: Practitioners 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. RESULTS: 49 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). CONCLUSION: In IM patients, DH/BS is a marker of severity and is associated with SSc (scleromyositis).