2026-04-14T06:52:37Zhttps://recercat.cat/oai/request

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

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 Institut Català de la Salut [Pijnenburg L, Arnaud L] Service de rhumatologie, Centre de Référence des Maladies Auto-immunes Systémiques Rares Est Sud-Ouest Hôpitaux Universitaires de Strasbourg, Université de Strasbourg, Strasbourg, France. [Giannini M] Service de rhumatologie, Centre de Référence des Maladies Auto-immunes Systémiques Rares Est Sud-Ouest Hôpitaux Universitaires de Strasbourg, Université de Strasbourg, Strasbourg, France. Exploration fonctionnelle musculaire, Service de Physiologie Explorations Fonctionnelles Musculaires, Hôpitaux Universitaires de Strasbourg, Université de Strasbourg, Strasbourg, France. [Bouchard-Marmen M] Division of Rheumatology, CHU de Quebec-Universite Laval, Quebec, Quebec, Canada. [Barsotti S] Department of Rheumatology and Clinical Immunology Unit, University of Pisa, Pisa, Italy. [Bellando-Randone S] Department of Experimental and Clinical Medicine, Universitaria Careggi, Azienda Ospedaliera, Florence, Italy. [Selva-O'Callaghan A] Servei de Medicina Interna, Vall d’Hebron Hospital Universitari, Barcelona, Spain Vall d'Hebron Barcelona Hospital Campus 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). 2023-09-19T08:31:24Z 2023-09-19T08:31:24Z 2023-08 info:eu-repo/semantics/article info:eu-repo/semantics/publishedVersion Pijnenburg L, Giannini M, Bouchard-Marmen M, Arnaud L, Barsotti S, Bellando-Randone S, et al. In inflammatory myopathies, dropped head/bent spine syndrome is associated with scleromyositis: an international case-control study. RMD Open. 2023 Aug;9(3):e003081. 2056-5933 https://hdl.handle.net/11351/10287 37666644 10.1136/rmdopen-2023-003081 http://hdl.handle.net/11351/10287 eng 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 application/pdf BMJ Scientia