ArticleArthritis care & research2014
Early illness features associated with mortality in the juvenile idiopathic inflammatory myopathies.
Article in Arthritis care & research, 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 41 papers, 1 of them a synthesis that pooled it.
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Who cites it
41 citing papers in PubMed, 1 synthesis or guideline pooled it, 90 citations in OpenAlex.
- British Society for Rheumatology guideline on management of paediatric, adolescent and adult patients with idiopathic inflammatory myopathy.Rheumatology (Oxford, England) · 2022Guideline
- Juvenile Myositis: Insights on Classification, Pathogenesis, and Therapies Over the Past 50 Years.Arthritis care & research · 2026Review
- 3D Nanofibrous Scaffolds for Encapsulation-Controlled Vancomycin Delivery: Antibacterial Performance and Cytocompatibility.Polymers · 2025Article
- Rituximab for Early-Onset Juvenile Dermatomyositis Complicated by Interstitial Lung Disease.Case reports in pediatrics · 2025Article
- Advances in Juvenile Dermatomyositis: Pathophysiology, Diagnosis, Treatment and Interstitial Lung Diseases-A Narrative Review.Children (Basel, Switzerland) · 2024Review
- Myositis-Associated Autoantibodies in Patients With Juvenile Myositis Are Associated With Refractory Disease and Mortality.Arthritis & rheumatology (Hoboken, N.J.) · 2024Article
- Rapidly progressive interstitial lung disease combined with pneumocystis jiroveci pneumonia in a patient with single anti-TIF-1γ antibody positive dermatomyositis in the context of an underlying tumor.BMC pulmonary medicine · 2023Article
- Juvenile idiopathic inflammatory myositis: an update on pathophysiology and clinical care.Nature reviews. Rheumatology · 2023Review
- Anti-FHL1 autoantibodies in juvenile myositis are associated with anti-Ro52 autoantibodies but not with severe disease features.Rheumatology (Oxford, England) · 2023Article
- Baseline characteristics of children with juvenile dermatomyositis enrolled in the first year of the new Childhood Arthritis and Rheumatology Research Alliance registry.Pediatric rheumatology online journal · 2022Article
- Association of anti-HSC70 autoantibodies with cutaneous ulceration and severe disease in juvenile dermatomyositis.Rheumatology (Oxford, England) · 2022Article
- Juvenile Dermatomyositis Magnetic Resonance Imaging Score (JIS) does not correlate with criteria for clinically inactive disease: a single-centre retrospective evaluation.Rheumatology international · 2022Article
- Cardiovascular Abnormalities in Juvenile Dermatomyositis: A Scoping Review for the Clinical Rheumatologists.Frontiers in medicine · 2022Article
- Updates on Juvenile Dermatomyositis from the Last Decade: Classification to Outcomes.Rheumatic diseases clinics of North America · 2021Review
- Severe onset of inflammatory myositis in a child: think to paraneoplastic myositis.Italian journal of pediatrics · 2021Article
- Corticosteroid discontinuation, complete clinical response and remission in juvenile dermatomyositis.Rheumatology (Oxford, England) · 2021Article
- Anti-MDA5 autoantibodies associated with juvenile dermatomyositis constitute a distinct phenotype in North America.Rheumatology (Oxford, England) · 2021Article
- Machine Learning in Rheumatic Diseases.Clinical reviews in allergy & immunology · 2021Review
- Juvenile dermatomyositis: advances in clinical presentation, myositis-specific antibodies and treatment.World journal of pediatrics : WJP · 2020Review
- Long-term outcomes in Juvenile Myositis patients.Seminars in arthritis and rheumatism · 2020Article
Corrections and comments
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Authors and funding
9 authors at 4 institutions in 2 countries.
Funding
Abstract
objectiveBecause juvenile idiopathic inflammatory myopathies (IIMs) are potentially life-threatening systemic autoimmune diseases, we examined risk factors for juvenile IIM mortality.
methodsMortality status was available for 405 patients (329 with juvenile dermatomyositis [DM], 30 with juvenile polymyositis [PM], and 46 with juvenile connective tissue disease-associated myositis [CTM]) enrolled in nationwide protocols. Standardized mortality ratios (SMRs) were calculated using US population statistics. Cox regression analysis was used to assess univariable associations with mortality, and random survival forest (RSF) classification and Cox regression analysis were used for multivariable associations.
resultsOf 17 deaths (4.2% overall mortality), 8 (2.4%) were in juvenile DM patients. Death was related to the pulmonary system (primarily interstitial lung disease [ILD]) in 7 patients, gastrointestinal system in 3, and multisystem in 3, and of unknown etiology in 4 patients. The SMR for juvenile IIMs overall was 14.4 (95% confidence interval [95% CI] 12.2-16.5) and was 8.3 (95% CI 6.4-10.3) for juvenile DM. The top mortality risk factors in the univariable analysis included clinical subgroup (juvenile CTM, juvenile PM), antisynthetase autoantibodies, older age at diagnosis, ILD, and Raynaud's phenomenon at diagnosis. In multivariable analyses, clinical subgroup, illness severity at onset, age at diagnosis, weight loss, and delay to diagnosis were the most important predictors from RSF; clinical subgroup and illness severity at onset were confirmed by multivariable Cox regression analysis.
conclusionOverall mortality was higher in juvenile IIM patients, and several early illness features were identified as risk factors. Clinical subgroup, antisynthetase autoantibodies, older age at diagnosis, and ILD are also recognized as mortality risk factors in adult myositis.
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