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- Title
Anti-SAE autoantibody in dermatomyositis: original comparative study and review of the literature.
- Authors
Demortier, Juliette; Vautier, Mathieu; Chosidow, Olivier; Gallay, Laure; Bessis, Didier; Berezne, Alice; Cordel, Nadège; Schmidt, Jean; Smail, Amar; Duffau, Pierre; Jachiet, Marie; Begon, Edouard; Gottlieb, Jeremy; Chasset, François; Graveleau, Julie; Marque, Myriam; Cesbron, Elise; Forestier, Amandine; Josse, Séverine; Kluger, Nicolas
- Abstract
Objective Among specific autoantibodies in DM, the anti–small ubiquitin-like modifier activating enzyme (SAE) antibody is rare. We aim to describe the clinical characteristics, cancer prevalence, and muscle pathology of anti-SAE–positive DM. Methods Patients with a diagnosis of DM and sera positive for the anti-SAE antibody were recruited from 19 centres in this retrospective observational study. The available muscular biopsies were reviewed. We conducted a comparison with anti-SAE–negative DM and a review of the literature. Results Of the patients in the study (n = 49), 84% were women. Skin involvement was typical in 96% of patients, with 10% having calcinosis, 18% ulceration and 12% necrosis; 35% presented with a widespread skin rash. Muscular disease affected 84% of patients, with mild weakness [Medical Research Council (MRC) scale 4 (3, 5)], although 39% of patients had dysphagia. Muscular biopsies showed typical DM lesions. Interstitial lung disease was found in 21% of patients, mainly with organizing pneumonia pattern, and 26% of patients showed dyspnoea. Cancer-associated myositis was diagnosed in 16% of patients and was responsible for the majority of deaths, its prevalence being five times that of the general population. IVIG therapy was administered to 51% of the patients during the course of the disease. Comparison with anti-SAE–negative DM (n = 85) showed less and milder muscle weakness (P = 0.02 and P = 0.006, respectively), lower creatinine kinase levels (P < 0.0001) and less dyspnoea (P = 0.003). Conclusion Anti-SAE positive DM is a rare subgroup associated with typical skin features but a potentially diffuse rash, a mild myopathy. Interstitial lung disease defines an organizing pneumonia pattern. Cancer associated DM prevalence is five times that of the general population. Trial registration ClinicalTrials.gov, http://clinicaltrials.gov , NCT04637672.
- Subjects
TUMOR risk factors; AUTOANTIBODIES; MUSCLE diseases; DERMATOMYOSITIS; SCIENTIFIC observation; BIOPSY; MUSCLES; RETROSPECTIVE studies; ACQUISITION of data; EXANTHEMA; INTERSTITIAL lung diseases; COMPARATIVE studies; DYSPNEA; INTRAVENOUS immunoglobulins; MUSCLE weakness; RISK assessment; DISEASE prevalence; MEDICAL records; DESCRIPTIVE statistics; CALCINOSIS; MYOSITIS; ENZYME inhibitors; NECROSIS; DISEASE complications
- Publication
Rheumatology, 2023, Vol 62, Issue 12, p3932
- ISSN
1462-0324
- Publication type
Article
- DOI
10.1093/rheumatology/kead154