Inaugural dropped head syndrome and camptocormia in inflammatory myopathies: a retrospective study

• Published in: Rheumatology (Oxford, England) Vol. 63; no. 2; pp. 506 - 515
• Main Authors: Robert, Marie, Lessard, Lola E R, Bouhour, Françoise, Petiot, Philippe, Fenouil, Tanguy, Svahn, Juliette, Fiscus, Julie, Fabien, Nicole, Perard, Laurent, Robinson, Philip, Durieu, Isabelle, Coury, Fabienne, Streichenberger, Nathalie, Hot, Arnaud, Gallay, Laure
• Format: Journal Article
• Language: English
• Published: England Oxford University Press 01.02.2024; Oxford University Press (OUP)
• Subjects: Dropped Head Syndrome; Female; Human health and pathology; Humans; Life Sciences; Male; Muscular Atrophy, Spinal - complications; Myositis - complications; Retrospective Studies; Rhumatology and musculoskeletal system; Santé publique et épidémiologie; Spinal Curvatures; inflammatory myopathies; dropped head syndrome; camptocormia; myositis
• ISSN: 1462-0324; 1462-0332; 1462-0332
• DOI: 10.1093/rheumatology/kead347

Objectives Inaugural axial muscle involvement, defined as dropped head syndrome (DHS) and/or camptocormia (CC), is poorly described in inflammatory myopathies (IM). This study aimed to further characterize IM patients with inaugural DHS/CC, their outcome and care management. Methods This retrospective study included IM patients diagnosed between 2000 and 2021. The main inclusion criterion was IM revealed by axial muscle deficit (DHS/CC). Results Twenty-seven patients were included; median (IQR) age at first symptoms was 66.0 years (55.5–75.0); 21 were female (77.8%). There were nine IBM, 33.3%, nine overlap myositis (OM, 33.3%), five DM, 18.5%, two immune checkpoint inhibitor-related myositis (7.4%), one focal myositis (3.7%) and one myositis with anti-Hu antibodies (3.7%). Age at first symptoms was ≤70 years in 16 patients (59.3%), including all DM patients and 8/9 OM patients (88.9%). In this group, partial remission of the disease was obtained in 9/16 (56.3%) and complete remission in 1/16 patients (6.3%); regression of DHS/CC was achieved in 3/16 patients (18.8%). Conversely, in the group of 11 patients aged >70 years at first symptoms, there were eight IBM (72.7%). Partial remission was obtained in 5/11 patients (45.5%), the disease was stable in 6/11 patients (54.5%); no complete remission was obtained nor regression of DHS/CC. Conclusion The analysis of IM patients with inaugural DHS/CC delineates two groups of patients according to the age at first symptoms in terms of clinical and outcome specificities, and proposes an adapted diagnostic and care management approach to prevent long-term complications.