2026-04-17T04:15:30Zhttps://recercat.cat/oai/request

oai:recercat.cat:11351/102872025-10-24T10:38:38Zcom_2072_378070com_2072_378040col_2072_378092

2025-10-24T10:38:38Z urn:hdl:11351/10287 In inflammatory myopathies, dropped head/bent spine syndrome is associated with scleromyositis: an international case–control study Pijnenburg, Luc Giannini, Margherita Bouchard Marmen, Maude ARNAUD, Laurent Barsotti, Simone Bellando-Randone, Silvia Selva-O'Callaghan, Albert Músculs - Malalties - Complicacions Esclerosi sistemàtica progressiva DISEASES::Musculoskeletal Diseases::Muscular Diseases::Myositis Other subheadings::Other subheadings::Other subheadings::/complications DISEASES::Skin and Connective Tissue Diseases::Connective Tissue Diseases::Scleroderma, Systemic DISEASES::Musculoskeletal Diseases::Muscular Diseases::Muscle Weakness ENFERMEDADES::enfermedades musculoesqueléticas::enfermedades musculares::miositis Otros calificadores::Otros calificadores::Otros calificadores::/complicaciones ENFERMEDADES::enfermedades de la piel y tejido conjuntivo::enfermedades del tejido conjuntivo::esclerodermia sistémica ENFERMEDADES::enfermedades musculoesqueléticas::enfermedades musculares::debilidad muscular Dermatomyositis; Polymyositis; Systemic sclerosis Dermatomiositis; Polimiositis; Esclerosis sistémica Dermatomiositis; Polimiositis; Esclerosi sistèmica 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). 2025-10-24T10:38:38Z 2025-10-24T10:38:38Z 2023-09-19T08:31:24Z 2023-09-19T08:31:24Z 2023-08 info:eu-repo/semantics/article info:eu-repo/semantics/publishedVersion http://hdl.handle.net/11351/10287 RMD Open;9(3) http://dx.doi.org/10.1136/rmdopen-2023-003081 Attribution-NonCommercial 4.0 International http://creativecommons.org/licenses/by-nc/4.0/ info:eu-repo/semantics/openAccess BMJ Scientia