ArticleRheumatology (Oxford, England)2021
Anti-MDA5 autoantibodies associated with juvenile dermatomyositis constitute a distinct phenotype in North America.
Article in Rheumatology (Oxford, England), 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 32 papers.
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Who cites it
32 citing papers in PubMed, 51 citations in OpenAlex.
- Juvenile Myositis: Insights on Classification, Pathogenesis, and Therapies Over the Past 50 Years.Arthritis care & research · 2026Review
- MDA5-associated juvenile dermatomyositis and interstitial lung disease from rapidly progressive to silent: a report of three cases in South African children and a review of the literature.Clinical rheumatology · 2026Review
- Anti-Melanoma Differentiation-Associated Gene 5 Antibody-Positive Juvenile Dermatomyositis Presenting With Predominant Joint Contractures.Revista de neurologia · 2026Article
- Use of nailfold capillaroscopy for evaluation of disease activity in juvenile dermatomyositis: Results of a two-center retrospective study.World journal of clinical pediatrics · 2026Article
- How loss-of-function mutations inJournal of translational autoimmunity · 2026Review
- Regional differences in clinical manifestations of antisynthetase syndrome: a comparison between Asian and European cohorts.Arthritis research & therapy · 2026Article
- Dermatomyositis: Prevalence, Clinical Spectrum, Diagnostic Approach, and Management Strategies.Archives of internal medicine research · 2026Article
- Application of HRCT-based radiomics to predict interstitial lung disease for juvenile dermatomyositis.BMC pediatrics · 2025Article
- Anti-melanoma differentiation-associated gene 5 antibody associated rapidly progressive interstitial lung disease in a pediatric patient: a case report.Journal of medical case reports · 2025Article
- Profile of patients with Juvenile Dermatomyositis and Anti-MDA5 autoantibodies.Pediatric research · 2025Article
- Antimelanoma differentiation-associated gene 5 (anti-MDA5) antibody-positive dermatomyositis: clinical features and outcomes in a racially diverse patient cohort.BMC rheumatology · 2025Article
- An update on autoantibodies in the idiopathic inflammatory myopathies.Nature reviews. Rheumatology · 2025Review
- Achieving Medication-Free Remission in Patients With Juvenile Dermatomyositis.ACR open rheumatology · 2025Article
- Juvenile Dermatomyositis: Updates in Pathogenesis and Biomarkers, Current Treatment, and Emerging Targeted Therapies.Paediatric drugs · 2025Review
- Advances in Juvenile Dermatomyositis: Pathophysiology, Diagnosis, Treatment and Interstitial Lung Diseases-A Narrative Review.Children (Basel, Switzerland) · 2024Review
- Cutaneous Ulcers and Response to Treatment in a Child with Anti-MDA5 Dermatomyositis.Mediterranean journal of rheumatology · 2024Article
- Treatment of myopathy and cutaneous ulcers in anti-MDA5-positive dermatomyositis with triple therapy.BMJ case reports · 2024Article
- Elevated liver enzymes: unusual presentation of anti-MDA5 antibody-associated juvenile dermatomyositis.Clinical and experimental rheumatology · 2024Article
- Clinical nomogram assisting in discrimination of juvenile dermatomyositis-associated interstitial lung disease.Respiratory research · 2023Article
- Juvenile idiopathic inflammatory myositis: an update on pathophysiology and clinical care.Nature reviews. Rheumatology · 2023Review
Corrections and comments
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Authors and funding
9 authors at 5 institutions in 2 countries.
Funding
Abstract
objectiveMyositis-specific autoantibodies have defined distinct phenotypes of patients with juvenile myositis (JIIM). We assessed the frequency and clinical significance of anti-melanoma differentiation-associated gene 5 (MDA5) autoantibody-associated JIIM in a North American registry.
methodsRetrospective examination of the characteristics of 35 JIIM patients with anti-MDA5 autoantibodies was performed, and differences from other myositis-specific autoantibody groups were evaluated.
resultsAnti-MDA5 autoantibodies were present in 35/453 (7.7%) of JIIM patients and associated with older age at diagnosis, and lower serum creatine kinase and aldolase levels. Patients with anti-MDA5 autoantibodies had more frequent weight loss, adenopathy, arthritis, interstitial lung disease (ILD), and less frequent falling compared with anti-transcriptional intermediary factor 1 (TIF1), anti-nuclear matrix protein 2 (NXP2) and myositis-specific autoantibody/myositis-associated autoantibody-negative patients. They had a different season of diagnosis and less frequent mechanic's hands and ILD compared with those with anti-synthetase autoantibodies. Anti-MDA5 patients received fewer medications compared with anti-TIF1, and corticosteroid treatment was shorter compared with anti-TIF1 and anti-nuclear matrix protein 2 autoantibody groups. The frequency of remission was higher in anti-MDA5 than anti-synthetase autoantibody-positive JIIM. In multivariable analyses, weight loss, arthritis and arthralgia were most strongly associated with anti-MDA5 autoantibody-positive JIIM.
conclusionAnti-MDA5 JIIM is a distinct subset, with frequent arthritis, weight loss, adenopathy and less severe myositis, and is also associated with ILD. Anti-MDA5 is distinguished from anti-synthetase autoantibody-positive JIIM by less frequent ILD, lower creatine kinase levels and differing seasons of diagnosis. Anti-MDA5 has comparable outcomes, but with the ability to discontinue steroids more rapidly and less frequent flares compared with anti-TIF1 autoantibodies, and more frequent remission compared with anti-synthetase JIIM patients.
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