{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,22]],"date-time":"2026-09-22T10:40:12Z","timestamp":1790073612226,"version":"4.0.1"},"reference-count":21,"publisher":"BMJ","issue":"3","license":[{"start":{"date-parts":[[2023,9,4]],"date-time":"2023-09-04T00:00:00Z","timestamp":1693785600000},"content-version":"unspecified","delay-in-days":3,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["RMD Open"],"accepted":{"date-parts":[[2023,6,22]]},"published-print":{"date-parts":[[2023,9]]},"abstract":"<jats:sec>\n                  <jats:title>Background<\/jats:title>\n                  <jats:p>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).<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Objectives<\/jats:title>\n                  <jats:p>To assess the significance of DH\/BS in patients with IM.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Methods<\/jats:title>\n                  <jats:p>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.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Results<\/jats:title>\n                  <jats:p>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&lt;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\u22640.0005), 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&lt;0.0001). Distribution of the myopathy, its severity and its association with SSc were independently associated with DH\/BS (p&lt;0.05). Mortality was higher in the DH\/BS-IM patients and loss of walking ability was independently associated with survival (p&lt;0.05).<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Conclusion<\/jats:title>\n                  <jats:p>In IM patients, DH\/BS is a marker of severity and is associated with SSc (scleromyositis).<\/jats:p>\n               <\/jats:sec>","DOI":"10.1136\/rmdopen-2023-003081","type":"journal-article","created":{"date-parts":[[2023,9,4]],"date-time":"2023-09-04T12:25:40Z","timestamp":1693830340000},"page":"e003081","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":11,"title":["In inflammatory myopathies, dropped head\/bent spine syndrome is associated with scleromyositis: an international case\u2013control study"],"prefix":"10.1136","volume":"9","author":[{"given":"Luc","family":"Pijnenburg","sequence":"first","affiliation":[{"name":"Service de rhumatologie, Centre de R\u00e9f\u00e9rence des Maladies Auto-immunes Syst\u00e9miques Rares Est Sud-Ouest H\u00f4pitaux Universitaires de Strasbourg, Universit\u00e9 de Strasbourg, Strasbourg, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Margherita","family":"Giannini","sequence":"additional","affiliation":[{"name":"Service de rhumatologie, Centre de R\u00e9f\u00e9rence des Maladies Auto-immunes Syst\u00e9miques Rares Est Sud-Ouest H\u00f4pitaux Universitaires de Strasbourg, Universit\u00e9 de Strasbourg, Strasbourg, France"},{"name":"Exploration fonctionnelle musculaire, Service de Physiologie Explorations Fonctionnelles Musculaires, H\u00f4pitaux Universitaires de Strasbourg, Universit\u00e9 de Strasbourg, Strasbourg, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maude","family":"Bouchard-Marmen","sequence":"additional","affiliation":[{"name":"Division of Rheumatology, CHU de Quebec-Universite Laval, Quebec, Quebec, Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8077-8394","authenticated-orcid":false,"given":"Laurent","family":"Arnaud","sequence":"additional","affiliation":[{"name":"Service de rhumatologie, Centre de R\u00e9f\u00e9rence des Maladies Auto-immunes Syst\u00e9miques Rares Est Sud-Ouest H\u00f4pitaux Universitaires de Strasbourg, Universit\u00e9 de Strasbourg, Strasbourg, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Simone","family":"Barsotti","sequence":"additional","affiliation":[{"name":"Department of Rheumatology and Clinical Immunology Unit, University of Pisa, Pisa, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Silvia","family":"Bellando-Randone","sequence":"additional","affiliation":[{"name":"Department of Experimental and Clinical Medicine, Universitaria Careggi, Azienda Ospedaliera, Florence, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Livio","family":"Bernardi","sequence":"additional","affiliation":[{"name":"Departement of rheumatology, University of Padova, Padova, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paola","family":"Bini","sequence":"additional","affiliation":[{"name":"Department of neurology, Neurological Institute Mondino, University of Pavia, Pavia, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jelena","family":"Blagojevic","sequence":"additional","affiliation":[{"name":"Department of Experimental and Clinical Medicine, Universitaria Careggi, Azienda Ospedaliera, Florence, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2557-8514","authenticated-orcid":false,"given":"Veronica","family":"Codullo","sequence":"additional","affiliation":[{"name":"Rheumatology Division, University and IRCCS Policlinico S. 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