Observational studyClinical and experimental rheumatology2023
Anti-MDA5-positive dermatomyositis and remission in a single referral centre population.
Observational study in Clinical and experimental rheumatology, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 8 papers.
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Who cites it
8 citing papers in PubMed, 16 citations in OpenAlex.
- Clinical characteristics and outcomes of anti-MDA5 dermatomyositis: a retrospective study.BMC rheumatology · 2025Article
- Article
- The clinical features and mortality predictors for idiopathic inflammatory myopathies: a retrospective study of 572 patients and the "AIRMT" Score.Clinical rheumatology · 2025Article
- Epidemiology of myositis.Current opinion in rheumatology · 2025Review
- An update on autoantibodies in the idiopathic inflammatory myopathies.Nature reviews. Rheumatology · 2025Review
- [A unicenter real-world study of the correlation factors for complete clinical response in idiopathic inflammatory myopathies].Beijing da xue xue bao. Yi xue ban = Journal of Peking University. Health sciences · 2024Article
- MDA5-positive dermatomyositis without muscle weakness and rash: a case report and literature review.Frontiers in medicine · 2024Article
- Overlap Syndrome Consisting of Polymyositis/Dermatomyositis and ANCA-Associated Vasculitis According to the 2022 ACR/EULAR Criteria for Vasculitis: A Korean Single-Centre Study.Journal of clinical medicine · 2023Article
Corrections and comments
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Authors and funding
11 authors at 2 institutions in 1 country.
Funding
Abstract
objectivesTo describe a single-centre North American adult cohort of anti-MDA5-positive dermatomyositis patients, with emphasis on drug-free long-term remission.
methodsWe conducted an observational retrospective cohort study of anti-MDA5-positive DM patients. All consented patients seen in the Johns Hopkins Myositis Centre from 2003-2020 with suspected muscle disease were routinely screened for myositis-specific autoantibodies. All sera were screened for anti-MDA5 autoantibodies by line blot; positives were verified by enzyme-linked immunoassay. Patients whose sera were anti-MDA5 positive by both assays (n=52) were followed longitudinally. If clinical status was unavailable, structured telephone interviews were conducted. Clinical remission was defined as being off all immunosuppression >1 year while remaining asymptomatic.
results38/52 (73%) of the patients were women with a median age at disease-onset of 47 (IQR 40-54). Twenty-five of the patients (48%) were White, 16 (30%) were Black and 3 (6%) were Asian. Most patients (42/52, 80%) had interstitial lung disease, defined by inflammatory or fibrotic changes on high resolution computed tomography (HRCT). 18/52 (35%) of patients required pulse-dose methylprednisolone, 4/52 (8%) experienced spontaneous pneumothorax/pneumomediastinum, 6/52 (12%) required intubation, and 5/52 (10%) died. Over longitudinal follow-up (median 3.5 years), 9 (18%) patients achieved clinical remission. The median time from symptom onset to clinical remission was 4 years, and the median duration of sustained remission was 3.5 years (range 1.4-7.8). No demographic or disease characteristics were significantly associated with remission.
conclusionsIn this single centre, tertiary referral population of anti-MDA5-positive dermatomyositis, ~20% of patients experienced long-term drug-free remission after a median disease duration of 4 years. No clinical or biologic factors were associated with clinical remission.
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