In inflammatory myopathies, dropped head/bent spine syndrome is associated with scleromyositis: an international case-control study.
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Pijnenburg Luc, Giannini Margherita, Bouchard-Marmen Maude, Arnaud Laurent, Barsotti Simone, Bellando-Randone Silvia, Bernardi Livio, Bini Paola, Blagojevic Jelena, Codullo Veronica, Couderc Marion, De Moreuil Claire, Dernis Emanuelle, Diamanti Luca, Dubost Jean Jacques, Duval Fanny, Emmi Giacomo, Galempoix Jean-Marc, Geny Bernard, Gottenberg Jacques-Eric, Groza Monica, Guffroy Aurelien, Guichard Isabelle, Guilpain Philippe, Hervier Baptiste, Hudson Marie, Iaccarino Luca, Iannone Florenzo, Lebrun Delphine, Marchioni Enrico, Mariampillai Kuberaka, Maurier Francois, Mosca Marta, Nadaj-Pakleza Aleksandra, Nannini Carlotta, Piot Jean-Maxime, Prieto-González Sergio, Poursac Nicolas, Rouanet Eglantine, Sellam Jérémie, Selva-O'Callaghan Albert, Séverac François, Sibilia Jean, Sole Guilhem, Soulages Antoine, Terrier Benjamin, Tournadre Anne, Troyanov Yves, Vernier Nathalie, Vesperini Veronique, Viallard Jean-François, Ziane Rahima, Cavagna Lorenzo, Meyer Alain
Аннотация
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).