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Juvenile dermatomyositis (JDM) is the childhood-onset form of dermatomyositis, a systemic autoimmune inflammatory disorder characterized by proximal muscle weakness, characteristic skin changes, and multi-system inflammatory involvement. JDM is the most common idiopathic inflammatory myopathy in pediatric populations. Prevalence is not documented in this packet's structured fields. Patient advocacy and registry support is provided by the Myositis Support and Understanding Association, which maintains a patient registry, and the Myositis Support Group. No disease subtypes are documented in this packet's structured subtype field.
JDM is associated with 45 documented phenotypes in this packet spanning skin, muscle, and systemic domains. Very frequent features (80–99% of cases) include erythema, skin rash, calcinosis, myositis, myalgia, elevated circulating C-reactive protein concentration, and elevated erythrocyte sedimentation rate, reflecting both local tissue injury and systemic inflammatory activation. Frequent features (30–79% of cases) include poikiloderma, pruritus, cutaneous photosensitivity, arthritis, alopecia, fever, hypotonia, constipation, restrictive ventilatory defect, and vasculitis. The breadth of documented phenotypes reflects multi-system involvement spanning integumentary (skin), muscular, vascular, pulmonary, gastrointestinal, and articular domains. The affected_organ_systems field in this packet identifies skin and musculature as the primary involved systems; the phenotype data additionally documents systemic, vascular, pulmonary, and gastrointestinal involvement.
JDM is defined in this packet as a systemic autoimmune inflammatory condition. The known_genes and inheritance_patterns fields are not populated, consistent with an autoimmune rather than Mendelian genetic etiology. Specific immunological mechanisms underlying the inflammatory process in JDM, including autoantibody profiles or interferon pathway involvement, are not documented in this packet's certified structured fields.
Diagnostic criteria and methods are not documented in this packet's certified structured fields.
One FDA-approved therapy for dermatomyositis is documented in this packet. Octagam 10% (Immune Globulin Intravenous [Human]), marketed by Octapharma USA, carries FDA approval for the treatment of dermatomyositis. This approval covers intravenous immunoglobulin (IVIG) delivery for the indication. Additionally, umbilical cord lining stem cells (ULSC), under development by Restem LLC, hold an FDA orphan drug designation for treatment of idiopathic inflammatory myositis, including dermatomyositis and polymyositis. Orphan designation indicates the treatment is under development for a rare disease population and does not constitute regulatory approval for clinical use. No additional approved therapies are documented in this packet's structured fields.
14 trials found
Prognosis and natural history data are not documented in this packet's certified structured fields.
Four clinical trials in JDM and related inflammatory myopathies are documented in this packet, representing active investigation across pharmacologic and cellular therapy approaches. NCT07111065 — 'FAST for DM: Fatty Acid Supplementation Trial for Dermatomyositis,' sponsored by the National Institute of Environmental Health Sciences (NIEHS) — is a Phase 2 trial recruiting as of August 2026 with projected completion in November 2031. NCT07089121 — 'Descartes-08 for Children, Adolescents, and Young Adults With Autoimmune Disorders,' sponsored by Cartesian Therapeutics — is a Phase 1 trial recruiting since January 2026 with completion expected December 2028. NCT06154252 — 'RESET-Myositis: An Open-Label Study to Evaluate the Safety and Efficacy of CABA-201 in Subjects With Active Idiopathic Inflammatory Myopathy or Juvenile Idiopathic Inflammatory Myopathy,' sponsored by Cabaletta Bio — is a Phase 2 trial recruiting since December 2023 with completion planned for July 2028. NCT00059748 is a natural history and pathogenesis study of autoimmune disorders, currently recruiting. The research landscape for JDM comprises 226 classified publications, with case reports and review articles representing the dominant literature types alongside biomarker and gene therapy research, and with biologic and drug therapy interventions represented across the trial portfolio.
Data assembled from 5 of 12 sources · Last updated Sep 18, 2026, 1:12 PM UTC
Patient Advocacy Groups (PAGs) provide support, resources, and community for patients and caregivers.
European rare disease database
Genetic and Rare Diseases Info Center
AI-curated news mentioning juvenile dermatomyositis
Updated Sep 15, 2026
A new study presents clinical features and a nomogram designed to predict clinical response in juvenile dermatomyositis associated with interstitial lung disease. This research could enhance treatment strategies and patient outcomes in this rare condition.
A recent study highlights the diagnostic potential of interferon-stimulated gene 15 (ISG15) muscle expression in dermatomyositis. This research could enhance diagnostic accuracy for this rare autoimmune disease.
A systematic review highlights the complications of macrophage activation syndrome in juvenile dermatomyositis, providing insights into the disease's complexities. This research may inform future clinical approaches and management strategies.
Orsini will serve as the specialty pharmacy partner for Priovant's LISRAYA™ (brepocitinib), enhancing patient access to this innovative treatment. The drug was evaluated in the VALOR study, the largest placebo-controlled trial for dermatomyositis, indicating significant advancements in rare disease therapies.
Priovant Therapeutics secures FDA approval for its dual TYK2/JAK1 inhibitor, marking a significant milestone as the first approved treatment for adult dermatomyositis (DM). The approval is supported by data from the largest DM study ever conducted, the Valor trial, allowing use without restrictions based on disease activity or previous treatments.