Raras
Buscar doenças, sintomas, genes...
Dermatomiosite juvenil
ORPHA:93672CID-10 · M33.0CID-11 · 4A41.01PCDT · SUSDOENÇA RARA

A Dermatomiosite Juvenil (DMJ) é a forma de dermatomiosite (DM) que começa mais cedo na vida. É uma doença autoimune e inflamatória que afeta os músculos e o corpo todo. Ela se caracteriza por fraqueza nos músculos próximos ao tronco (como ombros e quadris), lesões ou manchas típicas na pele, e outros problemas que podem surgir em diferentes partes do corpo.

Mantido por Agente Raras·Colaborar como especialista →

Introdução

O que você precisa saber de cara

📋

A Dermatomiosite Juvenil (DMJ) é a forma de dermatomiosite (DM) que começa mais cedo na vida. É uma doença autoimune e inflamatória que afeta os músculos e o corpo todo. Ela se caracteriza por fraqueza nos músculos próximos ao tronco (como ombros e quadris), lesões ou manchas típicas na pele, e outros problemas que podem surgir em diferentes partes do corpo.

Pesquisas ativas
11 ensaios
41 total registrados no ClinicalTrials.gov
Publicações científicas
1.702 artigos
Último publicado: 2026 Apr 15

Escala de raridade

CLASSIFICAÇÃO ORPHANET · BRASIL 2024
Unknown
Ultra-rara
<1/50k
Muito rara
1/20k
Rara
1/10k
Pouco freq.
1/5k
Incomum
1/2k
Prevalência
0.0
Europe
Início
Adolescent
+ childhood, infancy
🏥
SUS: Cobertura mínimaScore: 30%
PCDT disponívelCID-10: M33.0
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Entender a doença

Do básico ao detalhe, leia no seu ritmo

Preparando trilha educativa...

Sinais e sintomas

O que aparece no corpo e com que frequência cada sintoma acontece

Partes do corpo afetadas

🧬
Pele e cabelo
6 sintomas
🫃
Digestivo
4 sintomas
🫁
Pulmão
3 sintomas
❤️
Coração
3 sintomas
💪
Músculos
3 sintomas
🧠
Neurológico
2 sintomas

+ 21 sintomas em outras categorias

Características mais comuns

90%prev.
Concentração elevada de proteína C-reativa circulante
Muito frequente (99-80%)
90%prev.
Autoimunidade
Muito frequente (99-80%)
90%prev.
Eritema
Muito frequente (99-80%)
90%prev.
Mialgia
Muito frequente (99-80%)
90%prev.
Concentração elevada de creatina quinase circulante
Muito frequente (99-80%)
90%prev.
Erupção cutânea
Muito frequente (99-80%)
45sintomas
Muito frequente (15)
Frequente (13)
Ocasional (17)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 45 características clínicas mais associadas, ordenadas por frequência.

Concentração elevada de proteína C-reativa circulanteElevated circulating C-reactive protein concentration
Muito frequente (99-80%)90%
AutoimunidadeAutoimmunity
Muito frequente (99-80%)90%
EritemaErythema
Muito frequente (99-80%)90%
MialgiaMyalgia
Muito frequente (99-80%)90%
Concentração elevada de creatina quinase circulanteElevated circulating creatine kinase concentration
Muito frequente (99-80%)90%

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico1.702PubMed
Últimos 10 anos200publicações
Pico2025120 papers
Linha do tempo
2026Hoje · 2026🧪 1995Primeiro ensaio clínico📈 2025Ano de pico
Publicações por ano (últimos 10 anos)

Encontrou um erro ou informação desatualizada? Sugira uma correção →

Genética e causas

O que está alterado no DNA e como passa nas famílias

🧬

Nenhum gene associado encontrado

Os dados genéticos desta condição ainda estão sendo catalogados.

Diagnóstico

Os sinais que médicos procuram e os exames que confirmam

Carregando...

Tratamento e manejo

Remédios, cuidados de apoio e o que precisa acompanhar

Pipeline de tratamentos
Pipeline regulatório — de medicamentos já aprovados a drogas em pesquisa exploratória.
Aprovado1
3Fase 32
2Fase 25
1Fase 13
·Pré-clínico9
Medicamentos catalogadosEnsaios clínicos· 0 medicamentos · 20 ensaios
Carregando informações de tratamento...

Onde tratar no SUS

Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)

🇧🇷 Atendimento SUS — Dermatomiosite juvenil

🗺️

Selecione um estado ou use sua localização para ver resultados.

Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.

Pesquisa ativa

Ensaios clínicos abertos e novidades científicas recentes

🟢 Recrutando agora

11 pesquisas recrutando participantes. Converse com seu médico sobre a possibilidade de participar.

Outros ensaios clínicos

41 ensaios clínicos encontrados, 11 ativos.

Distribuição por fase
Ver todos no ClinicalTrials.gov
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Publicações mais relevantes

📖Melhor nível de evidência: Revisão
Timeline de publicações
908 papers (10 anos)

Mostrando amostra de 200 publicações de um total de 908

#1

Reliability of durometry to assess firmness of calcinosis lesions in Juvenile and adult dermatomyositis.

PloS one2026

Dermatomyositis (DM) and juvenile dermatomyositis (JDM) are inflammatory myopathies affecting multiple organs, including muscle and skin. Calcinosis is a complication of DM/JDM that causes significant morbidity; however, few tools exist to assess calcinosis in DM/JDM patients This study aimed to evaluate the reliability of durometry measurements to assess the firmness of calcinosis lesions in DM and JDM patients. Calcinosis firmness was measured using a handheld digital durometer. Six investigators across 3 institutions examined DM/JDM calcinosis lesions by durometry, as well as control readings in healthy unaffected skin/subcutaneous tissue in similar anatomic areas, recording three readings per site. Intra-rater and inter-rater intraclass correlations were evaluated. We enrolled 57 patients and gathered 709 measurements (443 calcinosis lesions; 266 control lesions) over eleven anatomic regions. Intra-rater reliability was high across sites, while inter-rater reliability varied, being moderate to good in most areas, but poor in the thigh and anterior calf. Durometry readings were higher in calcinosis lesions than control sites overall. Measurements moderately correlated with qualitative physician assessments. Due to our study's cross-sectional nature, we could not assess calcinosis over time. Durometry is a novel, reliable, quantitative measure in assessing and characterizing targeted calcinosis lesions of DM/JDM patients.

#2

Intermittent Glucocorticoid Pulse Combined With Mycophenolate Mofetil in Juvenile Dermatomyositis.

JAMA dermatology2026 Jan 01

This cohort study reports the long-term clinical outcomes of the use of intermittent intravenous methylprednisolone pulse therapy combined with mycophenolate mofetil for patients with juvenile dermatomyositis.

#3

Presence of Multiple Muscle Specific Autoantibodies (MSAs) in a Young Child with Juvenile Dermatomyositis.

Indian journal of pediatrics2026 Feb 12
#4

Relapse of Juvenile Dermatomyositis following Eight Years in Remission: A Case Report.

Case reports in dermatology2026

Dermatomyositis is an autoimmune inflammatory condition that is characterized by proximal muscle weakness and a variety of cutaneous manifestations. In this report, we discuss the case of a 27-year-old white female who developed a relapse of juvenile dermatomyositis (JDM) 8 years after remission. The patient presented with a 4-month history of progressive proximal muscle weakness and rash, as well as a more recent onset of dysphagia. Severe cutaneous involvement was apparent on examination with ulcerated, pruritic plaques, violaceous papules, and diffuse erythema. Laboratories revealed elevated CK, aldolase, and CRP, with positive anti-NXP2, anti-U1 RNP, and anti-U2 RNP antibodies. Based on the clinical picture and diagnostic results, a relapse of JDM was diagnosed and various medication trials were initiated. Since initial presentation, the patient has improved and is currently stable on a regimen of hydroxychloroquine, IVIG, low-dose corticosteroids, tofacitinib, and gabapentin. Her myositis is in remission; however, she continues to experience burdensome cutaneous symptoms and overall significant disease impact. This case highlights the possibility of JDM relapse even after prolonged remission, underscoring the importance of maintaining a high index of suspicion for symptom recurrence. It also offers a thorough example of multimodal therapy use for refractory JDM.

#5

Severe Juvenile Dermatomyositis With Peripheral Nervous System Involvement: A Case Report.

Cureus2026 Feb

Juvenile dermatomyositis is the most common inflammatory myopathy of childhood. While many cases achieve favorable outcomes, severe forms may develop persistent disease activity, complications, and long-term disability. A five-year-old girl was admitted after 20 days of Gottron's papules and heliotrope rash, with intermittent fever during the first 10 days. She then developed mild proximal-predominant muscle weakness in all four limbs, rapidly progressing to severe generalized weakness with signs of axonal neuropathy over the subsequent 15 days. Despite aggressive immunosuppression with IV methotrexate, high-dose methylprednisolone pulses, IV immunoglobulin, oral prednisolone, and azathioprine, she demonstrated inadequate clinical response over four years of follow-up. Severe juvenile dermatomyositis with peripheral nervous system involvement may follow a refractory course despite guideline-directed aggressive immunosuppression. This case highlights the therapeutic challenges of rapidly progressive disease with atypical neurological complications and underscores the need for early recognition and intensified treatment strategies in high-risk presentations.

Publicações recentes

Ver todas no PubMed

📚 EuropePMC1.328 artigos no totalmostrando 199

2026

Novel use of anifrolumab in a young patient with Jo-1-positive juvenile dermatomyositis.

Rheumatology advances in practice
2026

Reliability of durometry to assess firmness of calcinosis lesions in Juvenile and adult dermatomyositis.

PloS one
2026

Relapse of Juvenile Dermatomyositis following Eight Years in Remission: A Case Report.

Case reports in dermatology
2026

Severe Juvenile Dermatomyositis With Peripheral Nervous System Involvement: A Case Report.

Cureus
2026

Global landscape of juvenile dermatomyositis research: a bibliometric analysis.

Pediatric research
2026

Comprehensive Analysis Reveals Potential Molecular Targets in Juvenile Dermatomyositis.

Biochemistry research international
2026

Nailfold video capillaroscopy as a non-invasive biomarker in juvenile dermatomyositis: A longitudinal analysis of microvascular changes and clinical relevance over one year.

Seminars in arthritis and rheumatism
2026

Distinct by Design: Unraveling the Unique Clinical and Transcriptomic Identity of Juvenile Scleromyositis Overlap Compared to Juvenile Systemic Sclerosis and Juvenile Dermatomyositis: Implications for Care and Pathogenesis.

ACR open rheumatology
2026

Transition From Juvenile Dermatomyositis to Spondyloarthritis: A Novel Overlapping Inflammatory Phenotype.

ACR open rheumatology
2026

Interferon-Driven Tryptophan Metabolism Links Inflammation and Mental Health in Juvenile Dermatomyositis.

Inflammation
2026

Presence of Multiple Muscle Specific Autoantibodies (MSAs) in a Young Child with Juvenile Dermatomyositis.

Indian journal of pediatrics
2026

Lung Involvement in MDA5-Positive Juvenile Dermatomyositis: A Case of Misdiagnosed Pneumonia in a Toddler.

Pediatric pulmonology
2026

Clinically validated assay for rapid determination of type I and type II interferon activity in systemic inflammatory diseases.

The Journal of allergy and clinical immunology
2026

Towards New Strategies: Case Report and Review of the Literature-Effective Use of JAK Inhibitor Baricitinib in a 4-Year-Old Boy with Anti-MDA5 Antibody-Positive Juvenile Dermatomyositis.

Journal of clinical medicine
2026

Comparison of disease severity and activity between juvenile- and adult-onset dermatomyositis: a multicentre cohort study.

Rheumatology (Oxford, England)
2026

An Unusual Case of Juvenile Dermatomyositis Complicated by Macrophage Activation Syndrome.

International journal of dermatology
2026

Comparison of pre- and post-natal environmental exposures in United States versus Brazilian patients with juvenile dermatomyositis.

Clinical and experimental rheumatology
2026

Sex Differences in PROMIS Pediatric Depressive Symptoms, Anxiety, and Psychological Stress Among Children and Adolescents Living With Pediatric Rheumatic Diseases.

The Journal of rheumatology
2026

Association of NXP2 autoantibodies with a more severe clinical phenotype of juvenile dermatomyositis.

Clinical and experimental rheumatology
2025

Unmasking inflammation in juvenile dermatomyositis: myokine profiles of patients and bioengineered human muscle.

Frontiers in immunology
2025

Rituximab for Early-Onset Juvenile Dermatomyositis Complicated by Interstitial Lung Disease.

Case reports in pediatrics
2025

A Comparative Bibliometric Analysis of Pediatric Interstitial Lung Disease Treatment: Global Trends, Advances, and Future Directions (2004-2024).

Pediatric pulmonology
2026

Juvenile dermatomyositis as a rare paraneoplastic manifestation of nasopharyngeal carcinoma.

Rheumatology (Oxford, England)
2025

Clinical use of intravenous immunoglobulin in juvenile dermatomyositis: indications, treatment course, and clinical outcomes.

Expert opinion on biological therapy
2026

MDA5 Antibody Associated Juvenile Dermatomyositis Presenting as Rapidly Progressive Pulmonary Fibrosis-Case Report.

Journal of paediatrics and child health
2026

Predictive role of muscle MRI in juvenile dermatomyositis: A tertiary center experience.

European journal of radiology
2025

Single-Cell Transcriptomics Reveals CCL3+ Classical Monocyte Subset Linked to Autoimmune Pathogenesis.

Journal of inflammation research
2025

Neutrophil lymphocyte ratio as a marker of skin disease activity in older children with juvenile dermatomyositis.

Pediatric rheumatology online journal
2025

Juvenile idiopathic inflammatory myopathies assessment by magnetic resonance imaging: a scoping review of protocols, scoring systems, and applications.

Advances in rheumatology (London, England)
2026

Intermittent Glucocorticoid Pulse Combined With Mycophenolate Mofetil in Juvenile Dermatomyositis.

JAMA dermatology
2026

Distinct cytokine signature in juvenile dermatomyositis: linking myositis-specific antibodies and clinical manifestations.

Cytokine
2025

Juvenile dermatomyositis: new insights into pathogenesis and clinical applications.

Pediatric rheumatology online journal
2025

[Clinical characteristics of juvenile dermatomyositis in anti-nuclear matrix protein 2 antibody-positive patients and risk factors for severity: a national multicenter retrospective study].

Zhonghua er ke za zhi = Chinese journal of pediatrics
2025

Superimposed Segmental Juvenile Dermatomyositis: A Case Report.

Cureus
2025

Toward a Treat-to-Target Strategy in Juvenile Dermatomyositis: What Are the Suitable Targets and Optimal Timing of Their Achievement?

Arthritis &amp; rheumatology (Hoboken, N.J.)
2025

Therapeutic plasma exchange in autoimmune diseases: a retrospective study in a tertiary pediatric hospital in Mexico.

Frontiers in pediatrics
2026

Congruence Between Child Self-Reported and Caregiver-Proxy-Reported Health-Related Quality of Life in Children With Juvenile Idiopathic Arthritis and Systemic Lupus Erythematosus.

The Journal of rheumatology
2025

Promising Treatments in Juvenile Dermatomyositis.

Rheumatic diseases clinics of North America
2025

Physical Activity Monitors in Myositis: Bridging the Gap from Bedside to Clinical Trials.

Rheumatic diseases clinics of North America
2025

Calcinosis in Adult and Juvenile Dermatomyositis: Pathogenesis and Potential Therapeutic Targets.

Rheumatic diseases clinics of North America
2025

Diverse Joint Manifestations in Anti-MDA5 Antibody-Positive Juvenile Dermatomyositis: A Unique Case Report.

International journal of rheumatic diseases
2025

Alternative polyadenylation and splicing regulation in refractory juvenile dermatomyositis: CELF2 at the crossroads.

Molecular therapy. Nucleic acids
2026

IFN-α levels correlate with muscle disease activity only in juvenile dermatomyositis patients with anti-MDA5+ autoantibodies.

Rheumatology (Oxford, England)
2025

Neutrophil activation in juvenile dermatomyositis: associations with muscle function and disease progression.

Pediatric rheumatology online journal
2025

Long-term outcomes of juvenile dermatomyositis.

Journal of the European Academy of Dermatology and Venereology : JEADV
2026

Validation of the 2019 EULAR/ACR classification criteria and clinical characteristics of childhood-onset Japanese patients with systemic lupus erythematosus.

Modern rheumatology
2025

The effect of Rituximab on B cells in pediatric autoimmune rheumatic diseases.

Northern clinics of Istanbul
2025

Is Hydroxychloroquine Useful for the Treatment of Cutaneous Manifestations of Idiopathic Inflammatory Myopathies? A Systematic Review.

Pharmaceuticals (Basel, Switzerland)
2025

Impaired Brachial Artery Reactivity in Young Adults With Clinically Quiescent Juvenile Dermatomyositis.

ACR open rheumatology
2026

PROMIS displays strong construct validity in pediatric and adult patients with idiopathic inflammatory myopathies.

Rheumatology (Oxford, England)
2025

[Relationship between common myositis-specific antibodies and clinical features in children with juvenile dermatomyositis].

Zhongguo dang dai er ke za zhi = Chinese journal of contemporary pediatrics
2025

Tumoral Calcinosis of the Knee With Secondary Infection in a Patient With Juvenile Dermatomyositis: Management Approach and Pathophysiological and Therapeutic Review.

Cureus
2025

Anti-TIF1-Gamma Autoantibodies-Positive Juvenile Dermatomyositis Associated With Interstitial Lung Disease: A Case Report.

Cureus
2026

Dazukibart for the treatment of severe TIF1γ-positive juvenile dermatomyositis.

Journal of the European Academy of Dermatology and Venereology : JEADV
2025

Banker Type of Juvenile Dermatomyositis.

Indian journal of dermatology
2025

Discovery of tissue-specific proteomic signatures in juvenile dermatomyositis highlights pathways reflecting persistent disease activity, clinical heterogeneity, and myositis-specific autoantibody subtype.

Annals of the rheumatic diseases
2025

Vitamin D Status in Paraguayan Children With Autoimmune Diseases.

International journal of rheumatic diseases
2025

A possible role for immunogenetic factors in myositis developing after vaccination in the pre-covid-19 era.

Frontiers in immunology
2025

A retrospective natural history study in adult and juvenile patients with incident dermatomyositis and polymyositis using real world data.

Clinical rheumatology
2025

[Clinical characteristics of anti-melanoma differentiation associated gene 5 antibody-positive juvenile dermatomyositis].

Zhonghua er ke za zhi = Chinese journal of pediatrics
2025

Non-targeted immunosuppressive and immunomodulatory therapies for idiopathic inflammatory myopathies.

The Cochrane database of systematic reviews
2025

Spatial transcriptomic analysis of muscle biopsy from patients with treatment-naive juvenile dermatomyositis reveals mitochondrial abnormalities despite disease-related interferon-driven signature.

Annals of the rheumatic diseases
2025

Anasarca in Juvenile Dermatomyositis: An Uncommon Presentation.

Indian journal of pediatrics
2025

Level of CD4+ central memory T cells and its clinical correlation in childhood-onset systemic lupus erythematosus.

Lupus
2025

Calcinosis Universalis: An Atypical Presentation of Mi-2 Positive Juvenile Dermatomyositis - A Case-Based Review.

Mediterranean journal of rheumatology
2025

Application of HRCT-based radiomics to predict interstitial lung disease for juvenile dermatomyositis.

BMC pediatrics
2025

Targeted immunosuppressive and immunomodulatory therapies for idiopathic inflammatory myopathies.

The Cochrane database of systematic reviews
2026

Treatment outcomes in 63 cases of juvenile dermatomyositis-associated calcinosis.

Clinical and experimental rheumatology
2025

CD68 + macrophage infiltrations in muscle tissue of juvenile dermatomyositis associated with the course of the disease, not with the severity.

Clinical rheumatology
2025

Updates in juvenile dermatomyositis: pathogenesis and therapy.

Current opinion in rheumatology
2025

Nailfold videocapillaroscopy in juvenile dermatomyositis: detailed correlations between microvascular abnormalities with clinical and laboratory parameters from an observational retrospective single-center study.

Clinical rheumatology
2025

Transition of CELF2 PAS usage promotes recovery of refractory JDM through alternative splicing regulation of CTSB.

Molecular therapy. Nucleic acids
2025

Juvenile dermatomyositis in Oman: clinical patterns and disease trajectory from a National cohort.

Pediatric rheumatology online journal
2026

Anifrolumab in Refractory Juvenile Dermatomyositis.

Pediatric dermatology
2025

Remission and low disease activity definitions in adult idiopathic inflammatory myopathies: A narrative review by myositis clinical trials consortium (MCTC).

Autoimmunity reviews
2025

Forty-one Cytokine Profile and Interferon-I Score in Juvenile Dermatomyositis: A Case Series Study.

Recent advances in inflammation &amp; allergy drug discovery
2025

Panniculitis in Anti-MDA-5 Positive Juvenile Dermatomyositis: A Case Report.

JNMA; journal of the Nepal Medical Association
2025

Juvenile dermatomyositis: a severe and atypical presentation.

ARP rheumatology
2025

Efficacy of hydroxychloroquine treatment for the skin manifestations of juvenile dermatomyositis.

Modern rheumatology case reports
2025

CAR T cell therapy for children with rheumatic disease: the time is now.

Nature reviews. Rheumatology
2025

Epidemiology of Juvenile Dermatomyositis-What We Know and What More Is Needed?

International journal of rheumatic diseases
2025

Efficacy and safety of rhGH in juvenile dermatomyositis children with glucocorticoid-related growth failure.

Rheumatology (Oxford, England)
2025

Muscle Spatial Transcriptomic Reveals Heterogeneous Profiles in Juvenile Dermatomyositis and Persistence of Abnormal Signature After Remission.

Cells
2025

Long-term outcome of juvenile dermatomyositis associated with lipodystrophy: experience of a University hospital.

Anais brasileiros de dermatologia
2025

A novel presentation of calcinosis cutis of the scalp in adult dermatomyositis.

Dermatology online journal
2025

Oral JAK inhibitors for pediatric inflammatory skin disease.

Current opinion in pediatrics
2025

[Clinical analysis of 4 cases of juvenile dermatomyositis complicated with massive hemorrhage of digestive tract].

Zhonghua er ke za zhi = Chinese journal of pediatrics
2025

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
2025

Predictors of persistent active disease and the longitudinal development of organ damage, patient-reported outcomes and autoantibodies in long-term juvenile dermatomyositis.

RMD open
2025

Myositis-specific autoantibody subtypes are associated with response to Janus kinase inhibitors in patients with juvenile dermatomyositis.

Rheumatology (Oxford, England)
2025

Rheumatologic manifestations in children with underlying inborn errors of immunity.

BMC rheumatology
2025

Treating juvenile dermatomyositis to target: Paediatric Rheumatology European Society/Childhood Arthritis and Rheumatology Research Alliance-endorsed recommendations from an international task force.

Annals of the rheumatic diseases
2025

Tofacitinib monotherapy as maintenance treatment in juvenile dermatomyositis: a case report.

RMD open
2025

Effect of graded nursing and feeding guidance based on water swallow test combined with enteral nutrition support in juvenile dermatomyositis children with swallowing dysfunction.

African journal of reproductive health
2025

Exploring the Association of Sociodemographic Factors and Primary Diagnosis With Transition Readiness in Adolescents With Rheumatic Disease.

ACR open rheumatology
2025

Juvenile Dermatomyositis.

Journal of cutaneous medicine and surgery
2025

Why Recommended Visit Intervals Should Be Extracted When Conducting Longitudinal Analyses Using Electronic Health Record Data: Examining Visit Mechanism and Sensitivity to Assessment Not at Random.

Statistics in medicine
2025

Effects of Juvenile Idiopathic Inflammatory Myopathies on Growth, Development, and Maturation: A Systematic Review and Metaanalysis.

The Journal of rheumatology
2025

Targeting interferon responses in juvenile dermatomyositis: Siglec-1 as an in vitro biomarker for JAK inhibitor efficacy.

Rheumatology (Oxford, England)
2025

Differences in Organ Damage Based on Age at Onset in Idiopathic Inflammatory Myopathies: A Retrospective Multicenter MYKO Study.

Internal medicine (Tokyo, Japan)
2025

F18-FDG PET-CT Findings in Juvenile-Onset Polyarteritis Nodosa: A First Series and Literature Review.

Journal of clinical medicine
2025

Juvenile Dermatomyositis and Hepatitis B Viral Infection.

European journal of case reports in internal medicine
2025

CAR-T cell therapy for juvenile-onset autoimmune diseases: a promising future?

Arthritis research &amp; therapy
2025

Juvenile Dermatomyositis Triggered by Influenza B: A Case Report on Viral-Induced Autoimmunity.

Pediatric dermatology
2025

Long-term effectiveness and safety of baricitinib treatment on refractory or severe juvenile dermatomyositis.

Rheumatology (Oxford, England)
2025

Interferon Response Gene Score in Juvenile Dermatomyositis.

The Journal of rheumatology
2025

Cannabinoids in the management of refractory juvenile dermatomyositis: a patient's perspective.

Clinical and experimental dermatology
2025

Imaging assessment of calcinosis in juvenile dermatomyositis: a narrative review.

Pediatric rheumatology online journal
2025

Tofacitinib: a promising agent for the treatment of persistent rashes in juvenile dermatomyositis.

World journal of pediatrics : WJP
2025

Rapid clinical and transcriptomic response to anifrolumab in refractory anti-TIF1γ-positive juvenile dermatomyositis.

Annals of the rheumatic diseases
2025

Juvenile Dermatomyositis in a Two-Year-Old Yemeni Girl in a Resource-Limited Setting: A Case Report.

Cureus
2025

Involving young people in research investigating comorbidity associated with childhood-onset rheumatic disease: perspectives of a series of focus groups.

BMC rheumatology
2025

Physical Growth Dynamics of Children With Juvenile Dermatomyositis.

Cureus
2025

A Case of Juvenile Dermatomyositis Presenting with Inverse Gottron's Papules: A Case Report.

Case reports in dermatology
2025

Perspectives and experiences of parents of children with juvenile dermatomyositis: a semi-structured interview study.

Pediatric rheumatology online journal
2025

Monozygotic twins discordant for juvenile dermatomyositis: clinical, serological and gene expression studies.

Pediatric rheumatology online journal
2025

Imaging features of juvenile dermatomyositis complicated by pyomyositis in a 12-year-old boy: A case report.

Radiology case reports
2025

Physical activity and quality of life of children and adolescents with juvenile idiopathic arthritis, juvenile systemic lupus erythematosus and juvenile dermatomyositis during the COVID-19 pandemic.

Jornal de pediatria
2025

Type I interferon signature: a quantitative standardized method for clinical application.

Clinical and experimental immunology
2025

Monoclonal antibody targeting IFNβ for the treatment of NXP2-positive ulcerative juvenile dermatomyositis.

Pediatrics
2025

Pseudoangioedema as a presenting symptom of dermatomyositis with antinuclear matrix protein 2 autoantibodies.

JAAD case reports
2025

Correlation Between Interferon Response Gene Score and Disease Activity in Juvenile Dermatomyositis.

The Journal of rheumatology
2025

Tape strip expression profiling of juvenile dermatomyositis skin reveals mitochondrial dysfunction contributing to disease endotype.

JCI insight
2025

Effect of abatacept added to mycophenolate mofetil for refractory calcinosis in juvenile dermatomyositis.

Modern rheumatology case reports
2025

Successful treatment of an anti-MDA5 antibody-positive Juvenile Dermatomyositis patient with refractory interstitial lung disease using tofacitinib.

Pediatric rheumatology online journal
2025

Mortality in children and adolescents with autoimmune inflammatory rheumatic diseases admitted to the pediatric intensive care unit.

Pediatric rheumatology online journal
2025

[A case of vedolizumab treatment for refractory juvenile dermatomyositis with secondary intestinal perforation].

Zhonghua er ke za zhi = Chinese journal of pediatrics
2025

Psychometric properties of patient-reported outcomes measurement information system (PROMIS) fixed short forms in Juvenile Myositis.

Seminars in arthritis and rheumatism
2025

Approach to Janus kinase inhibition for juvenile dermatomyositis among CARRA and PReS providers.

Rheumatology (Oxford, England)
2024

Anti-nuclear matrix protein 2 antibody-associated juvenile dermatomyositis with gastrointestinal perforations was successfully treated with traditional therapeutic drugs combined with vedolizumab: a case report after a long-term follow-up and a review of the literature.

Frontiers in pediatrics
2025

Exploring the clinical profiles and management of juvenile dermatomyositis in Africa: a survey of African rheumatology care providers.

Pediatric rheumatology online journal
2024

Immunobiologics in juvenile dermatomyositis: a systematic review of promising therapeutic advances.

Reumatologia
2025

The Role of Pyroptosis-Related Gene Signature and Immune Infiltration in Juvenile Dermatomyositis.

Clinical, cosmetic and investigational dermatology
2024

Safety and Efficacy of Long-term Use of Infliximab in Severe Juvenile Dermatomyositis - 12 Years of Follow-up.

Acta dermatovenerologica Croatica : ADC
2025

Thromboembolic Risk in Juvenile Dermatomyositis Patients Treated With Intravenous Immunoglobulin.

Pediatric dermatology
2024

Anti-MDA5 Positive Juvenile Dermatomyositis With Macrophage Activation Syndrome: A Case Report.

Cureus
2025

Juvenile Dermatomyositis Associated Cholestatic Liver Disease With Normal Creatine Kinase.

Journal of paediatrics and child health
2025

Unforeseen complication in juvenile dermatomyositis.

Rheumatology (Oxford, England)
2025

Achieving Medication-Free Remission in Patients With Juvenile Dermatomyositis.

ACR open rheumatology
2024

The value of quantitative muscle ultrasound in monitoring muscle and fascia inflammatory activity in juvenile dermatomyositis patients.

Quantitative imaging in medicine and surgery
2025

Radiographs in Pediatric Rheumatology: Where Do We Stand?

The Indian journal of radiology &amp; imaging
2024

Transient negativity for the anti-TIF1γ antibody before treatment in juvenile dermatomyositis.

Rheumatology advances in practice
2025

A Bayesian latent class approach to causal inference with longitudinal data.

Statistical methods in medical research
2024

Successful tocilizumab treatment for rapidly progressive interstitial lung disease with anti-MDA5-positive juvenile dermatomyositis: a case report and literature review.

Frontiers in pediatrics
2025

25 Years of Biologics for the Treatment of Pediatric Rheumatic Disease: Advances in Prognosis and Ongoing Challenges.

Arthritis care &amp; research
2024

Case Report: IVIG causing bilateral papilledema and increased intracranial hypertension in patients with anti-TIF-1γ antibody-positive JDM.

Frontiers in pediatrics
2024

Anasarca and Systemic Capillary Leak Syndrome as an Unusual Presentation of Juvenile Dermatomyositis: Report of Three Cases and Case-Based Review.

International journal of rheumatic diseases
2025

Anti-lipoprotein lipase antibodies: A review.

Autoimmunity reviews
2024

Calcinosis universalis in juvenile dermatomyositis.

The Pan African medical journal
2025

Expert Perspective: Diagnostic Approach to Differentiating Juvenile Dermatomyositis From Muscular Dystrophy.

Arthritis &amp; rheumatology (Hoboken, N.J.)
2024

Anesthetic Challenges in Laparoscopic Surgery for a Child with Juvenile Dermatomyositis.

Cureus
2025

Recent Advances in Juvenile Dermatomyositis: Moving toward Integration of Myositis-Specific Antibody Clinical Phenotypes, IFN-Driven Pathogenesis, and Targeted Therapies.

The Journal of investigative dermatology
2025

Predictors of poor outcomes in juvenile dermatomyositis: what do we know? A narrative review.

Reumatismo
2024

Neutrophil and mononuclear leukocyte pathways and upstream regulators revealed by serum proteomics of adult and juvenile dermatomyositis.

Arthritis research &amp; therapy
2025

Rituximab Treatment in Adult Patients With Idiopathic Inflammatory Myositis: A Systematic Review and Meta-analysis.

Journal of clinical rheumatology : practical reports on rheumatic &amp; musculoskeletal diseases
2025

Anti-Sp4 and anti-CCAR1 autoantibodies in UK vs US patients with adult and juvenile-onset anti-TIF1γ-positive myositis.

Rheumatology (Oxford, England)
2024

Dyslipidemia in Juvenile Dermatomyositis.

Scientific reports
2024

Ringing in the Patient Voice: Measuring Patients' Lived Experience of Dermatomyositis Symptoms.

The Journal of rheumatology
2024

Genome-wide identification of cell type-specific susceptibility genes for Juvenile dermatomyositis through the analysis of N6-methyladenosine-associated SNPs.

Autoimmunity
2025

Interferon-β-Induced Injury During Pediatric Muscle Differentiation: Insight Into Juvenile Dermatomyositis Pathogenesis.

ACR open rheumatology
2025

Juvenile Dermatomyositis: Updates in Pathogenesis and Biomarkers, Current Treatment, and Emerging Targeted Therapies.

Paediatric drugs
2025

Epidemiological Characteristics of Patients With Juvenile Dermatomyositis in China: A Multicenter Study.

The Journal of rheumatology
2025

Longitudinal assessment of disease activity and muscle strength in juvenile dermatomyositis: a multicentre registry study.

Clinical and experimental rheumatology
2024

Penicillamine-Induced Juvenile Dermatomyositis: A Rare Clinical Encounter.

Indian dermatology online journal
2024

Rare skin manifestation of juvenile dermatomyositis: peri-orbital oedema and facial swelling.

Paediatrics and international child health
2024

Nailfold Capillaroscopy Changes in Patients with Idiopathic Inflammatory Myopathies.

Journal of clinical medicine
2024

Advances in Juvenile Dermatomyositis: Pathophysiology, Diagnosis, Treatment and Interstitial Lung Diseases-A Narrative Review.

Children (Basel, Switzerland)
2025

Profile of patients with Juvenile Dermatomyositis and Anti-MDA5 autoantibodies.

Pediatric research
2024

14-month-old female with anti-MDA5 juvenile dermatomyositis complicated by liver disease: a case report.

Pediatric rheumatology online journal
2024

Juvenile Dermatomyositis: Diagnosis and Management.

Skinmed
2025

Mining for Gems: Early Detection of Calcinosis in Juvenile Dermatomyositis Using Low-Dose Computed Tomography.

The Journal of rheumatology
2024

Double-negative T cells in pediatric rheumatic diseases.

Clinical and experimental pediatrics
2024

The influence of environmental factors related to Juvenile Dermatomyositis (JDM), its course and refractoriness to treatment.

Advances in rheumatology (London, England)
2024

Cutaneous Ulcers and Response to Treatment in a Child with Anti-MDA5 Dermatomyositis.

Mediterranean journal of rheumatology
2024

Atherosclerotic cardiovascular disease following a diagnosis of idiopathic inflammatory myopathy: analysis from a retrospective cohort in the TriNetX registry.

Clinical rheumatology
2024

Personalised medicine in juvenile dermatomyositis: From novel insights in disease mechanisms to changes in clinical practice.

Best practice &amp; research. Clinical rheumatology
2024

Paraneoplastic dermatomyositis and Hodgkin's lymphoma in a 14-year-old girl: a case report and literature review.

Frontiers in oncology
2024

Prevalence and Clinical Implications of Autoantibodies in Juvenile Dermatomyositis: A Single-Center Experience From India.

Journal of clinical rheumatology : practical reports on rheumatic &amp; musculoskeletal diseases
2024

Differences in the autoantibody phenotypes and long-term outcomes between juvenile- and adult-idiopathic inflammatory myopathies.

Seminars in arthritis and rheumatism
2024

Current evidence for janus kinase inhibitors in adult and juvenile dermatomyositis and key comparisons.

Expert opinion on pharmacotherapy
2024

Calcinosis in dermatomyositis.

Current opinion in rheumatology
2024

Increased vascular deposition of oxidized LDL in untreated juvenile dermatomyositis.

Pediatric rheumatology online journal
2024

Assay for interferon gamma release as a novel marker in pediatric patients with systemic lupus erythematosus.

Pediatric rheumatology online journal
2024

Decoding the genetic landscape of juvenile dermatomyositis: insights from phosphorylation-associated single nucleotide polymorphisms.

Immunogenetics
2024

Elicitation of expert prior opinion to design the BARJDM trial in juvenile dermatomyositis.

Rheumatology (Oxford, England)
2024

Over Activation of IL-6/STAT3 Signaling Pathway in Juvenile Dermatomyositis.

Rheumatology and therapy
2024

Juvenile dermatomyositis complications: navigating gastrointestinal perforations and treatment challenges, a case report.

Frontiers in pediatrics
2024

Clinical characteristics and prognostic factor in juvenile dermatomyositis: data of the Spanish registry.

Pediatric rheumatology online journal
2024

Capillaroscopy in the daily clinic of the pediatric rheumatologist.

Best practice &amp; research. Clinical rheumatology
2024

An Adolescent Case of Anti-MDA5 Antibody-Positive Juvenile Dermatomyositis With Interstitial Lung Disease Successfully Treated by Multitarget Therapy Avoiding Cyclophosphamide: A Case Report and Literature Review.

Cureus
2024

Autologous haematopoiesis stem cell transplantation (AHSCT) for treatment-refractory autoimmune diseases in children.

RMD open
2024

The Diagnostic Role of Skin Manifestations in Rheumatic Diseases in Children: A Critical Review of Paediatric Vasculitis.

International journal of molecular sciences
2024

Decreased Peripheral Blood Natural Killer Cell Count in Untreated Juvenile Dermatomyositis Is Associated with Muscle Weakness.

International journal of molecular sciences
2024

Familial clustering of dysbiotic oral and fecal microbiomes in juvenile dermatomyositis.

Scientific reports
2024

Undercover lung damage in pediatrics - a hot spot in morbidity caused by collagenoses.

Frontiers in immunology
2024

Increased percentage of HLA-DR T cells in untreated juvenile dermatomyositis.

Clinical immunology communications
2024

Case Report: Contiguous presentation of anti-MDA5 juvenile dermatomyositis and anti-AQP4 neuromyelitis optica spectrum disorder in an adolescent patient.

Frontiers in pediatrics
2024

Childhood Arthritis and Rheumatology Research Alliance Biologic Disease-Modifying Antirheumatic Drug Consensus Treatment Plans for Refractory Moderately Severe Juvenile Dermatomyositis.

Arthritis care &amp; research
2024

Autologous CD19-Targeting CAR T Cells in a Patient With Refractory Juvenile Dermatomyositis.

Arthritis &amp; rheumatology (Hoboken, N.J.)
Ver todos os 1.328 no EuropePMC

Associações

Organizações que acompanham esta doença — pra ter apoio e orientação

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Comunidades

Grupos ativos de quem convive com esta doença aqui no Raras

Ainda não existe comunidade no Raras para Dermatomiosite juvenil

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Doenças relacionadas

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Referências e fontes

Bases de dados externas citadas neste artigo

Publicações científicas

Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.

  1. Reliability of durometry to assess firmness of calcinosis lesions in Juvenile and adult dermatomyositis.
    PloS one· 2026· PMID 41871052mais citado
  2. Intermittent Glucocorticoid Pulse Combined With Mycophenolate Mofetil in Juvenile Dermatomyositis.
    JAMA dermatology· 2026· PMID 41259047mais citado
  3. Presence of Multiple Muscle Specific Autoantibodies (MSAs) in a Young Child with Juvenile Dermatomyositis.
    Indian journal of pediatrics· 2026· PMID 41678111mais citado
  4. Relapse of Juvenile Dermatomyositis following Eight Years in Remission: A Case Report.
    Case reports in dermatology· 2026· PMID 41868201mais citado
  5. Severe Juvenile Dermatomyositis With Peripheral Nervous System Involvement: A Case Report.
    Cureus· 2026· PMID 41859607mais citado
  6. Systematic literature review informing PReS/CARRA-endorsed recommendations for the treat-to-target strategy in juvenile dermatomyositis.
    J Rheumatol· 2026· PMID 41985922recente
  7. Introducing a parent-centred composite disease activity score for remote monitoring in juvenile dermatomyositis.
    Rheumatology (Oxford)· 2026· PMID 41978918recente
  8. Use of the EOS 2D/3D Imaging System for the Evaluation of Calcinosis in Juvenile Dermatomyositis: A Retrospective Case Series.
    J Clin Rheumatol· 2026· PMID 41973030recente
  9. Wong-type juvenile dermatomyositis associated with anti-MDA5 and anti-PL-7 antibodies.
    J Dtsch Dermatol Ges· 2026· PMID 41972486recente
  10. CAR T-cell therapy and indications for pediatric rheumatology: Structured overview of applications, risks, and considerations.
    Best Pract Res Clin Rheumatol· 2026· PMID 41927380recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:93672(Orphanet)
  2. MONDO:0008054(MONDO)
  3. Dermatomiosite e Polimiosite(PCDT · Ministério da Saúde)
  4. GARD:6805(GARD (NIH))
  5. Busca completa no PubMed(PubMed)
  6. Artigo Wikipedia(Wikipedia)
  7. Q6318950(Wikidata)

Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.

Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar

Dermatomiosite juvenil
Compêndio · Raras BR

Dermatomiosite juvenil

ORPHA:93672 · MONDO:0008054
🇧🇷 Brasil SUS
Geral
Prevalência
Unknown
Herança
Not applicable
CID-10
M33.0 · Dermatomiosite juvenil
CID-11
Ensaios
11 ativos
Início
Adolescent, Childhood, Infancy
Prevalência
0.0 (Europe)
MedGen
UMLS
C0263666
Repurposing
2 candidatos
potassium-p-aminobenzoateglucocorticoid receptor agonist
prednisone
EuropePMC
Wikidata
Wikipedia
Papers 10a
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