Raras
Buscar doenças, sintomas, genes...
Dermatomiosite
ORPHA:221CID-10 · M33.1CID-11 · 4A41.00PCDT · SUSDOENÇA RARA

A dermatomiosite (DM) é um tipo de miopatia inflamatória idiopática caracterizada por lesões cutâneas evocativas e fraqueza muscular proximal simétrica.

Mantido por Agente Raras·Colaborar como especialista →

Introdução

O que você precisa saber de cara

📋

A dermatomiosite (DM) é um tipo de miopatia inflamatória idiopática caracterizada por lesões cutâneas evocativas e fraqueza muscular proximal simétrica.

Pesquisas ativas
29 ensaios
184 total registrados no ClinicalTrials.gov
Publicações científicas
12.722 artigos
Último publicado: 2026

Escala de raridade

CLASSIFICAÇÃO ORPHANET · BRASIL 2024
1-9 / 100 000
Ultra-rara
<1/50k
Muito rara
1/20k
Rara
1/10k
Pouco freq.
1/5k
Incomum
1/2k
Prevalência
1.97
Australia
Início
Adult
+ elderly
🏥
SUS: Cobertura mínimaScore: 30%
PCDT disponívelCID-10: M33.1
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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
11 sintomas
💪
Músculos
8 sintomas
🫁
Pulmão
8 sintomas
❤️
Coração
7 sintomas
🫃
Digestivo
6 sintomas
🧠
Neurológico
2 sintomas

+ 47 sintomas em outras categorias

Características mais comuns

90%prev.
Edema
Muito frequente (99-80%)
90%prev.
Morfologia anormal da pálpebra
Muito frequente (99-80%)
90%prev.
Anormalidade no EMG
Muito frequente (99-80%)
90%prev.
Eritema
Muito frequente (99-80%)
90%prev.
Autoimunidade
Muito frequente (99-80%)
90%prev.
Fraqueza muscular da cintura escapular/pélvica
Muito frequente (99-80%)
97sintomas
Muito frequente (10)
Frequente (32)
Ocasional (34)
Muito raro (1)
Sem dados (20)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 97 características clínicas mais associadas, ordenadas por frequência.

Edema
Muito frequente (99-80%)90%
Morfologia anormal da pálpebraAbnormal eyelid morphology
Muito frequente (99-80%)90%
Anormalidade no EMGEMG abnormality
Muito frequente (99-80%)90%
EritemaErythema
Muito frequente (99-80%)90%
AutoimunidadeAutoimmunity
Muito frequente (99-80%)90%

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico12.722PubMed
Últimos 10 anos200publicações
Pico2026156 papers
Linha do tempo
2026Hoje · 2026🧪 1990Primeiro ensaio clínico
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.
3Fase 35
2Fase 23
1Fase 12
·Pré-clínico10
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

🗺️

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

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

NCT07111065 · FAST for DM - Fatty Acid Supplementation Trial (FAST) for De…Recrutando
PHASE2
NCT06455449 · A Study to Investigate the Efficacy and Safety of Anifroluma…Recrutando
PHASE3
NCT03816345 · Testing an Immunotherapy Anti-cancer Drug, Nivolumab, for Ad…Recrutando
PHASE1
NCT01276470 · Environmental Risk Factors for the Anti-synthetase SyndromeRecrutando
NCT06154252 · RESET-Myositis: An Open-Label Study to Evaluate the Safety a…Recrutando
PHASE2, PHASE3
NCT07089121 · Descartes-08 for Children, Adolescents, and Young Adults Wit…Recrutando
PHASE1, PHASE2
NCT06698796 · A Study to Understand How the Study Medicine Dazukibart Work…Recrutando
PHASE3
NCT00017914 · Adult and Juvenile MyositisRecrutando
NCT00059748 · Studies of the Natural History, Pathogenesis, and Outcome of…Recrutando
NCT05895786 · A Study to Understand How the Study Medicine (PF-06823859) W…Recrutando
PHASE3
NCT06371417 · Phase 1b Trial of RAY121 in Immunological Diseases (RAINBOW …Recrutando
PHASE1
NCT07160205 · Safety and Efficacy of ULSC on Disease Severity and Steroid …Recrutando
PHASE2, PHASE3
NCT02945345 · Clinical Responsiveness of Dermatomyositis Using Cutaneous D…Recrutando
NCT04402086 · Rheumatology Patient Registry and BiorepositoryRecrutando
NCT07403188 · A Long-Term Follow-Up Study for Participants Previously Trea…Recrutando
NCT07377058 · RCT of Tocilizumab for Anti-MDA5+DMRecrutando
NA
NCT07374107 · MIHRA - Patient-Rooted Insights for Shaping Myositis Science…Recrutando
NCT07345949 · Panniculitis in DermatomyositisRecrutando
NCT07265999 · Severity Factors of Dermatomyositis in the Caribbean Populat…Recrutando
NCT06857240 · Topical Ruxolitinib Cream for Refractory Cutaneous Dermatomy…Recrutando
PHASE2

Outros ensaios clínicos

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Publicações mais relevantes

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

Mostrando amostra de 200 publicações de um total de 5.814

#1

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

PloS one2026

A calcinose é uma complicação debilitante da dermatomiosite que carece de ferramentas de avaliação. Este estudo demonstra que a durômetria, que mede a firmeza das lesões, é um método novo e confiável para quantificar e caracterizar essas lesões. Isso oferece a médicos uma ferramenta objetiva e padronizada para monitorar a calcinose, potencialmente otimizando o manejo e o acompanhamento dos pacientes.

🇧🇷 traduzido
#2

Dermatomyositis masquerading as angioedema: a crucial differential not to overlook.

BMJ case reports2026 Mar 04

A dermatomiosite é uma doença autoimune rara que exige tratamento imunossupressor imediato para evitar danos multiorgânicos. Este artigo alerta que ela pode se manifestar de forma atípica, como inchaço grave no pescoço, mimetizando angioedema, o que pode atrasar o diagnóstico. É crucial que médicos considerem a dermatomiosite em casos de edema incomum, especialmente com alterações na pele e elevação da creatina quinase (CK), para garantir um tratamento urgente e eficaz.

🇧🇷 traduzido
#3

Spatial transcriptomics reveals mechanism of autoimmunity driven by internalized autoantibodies.

medRxiv : the preprint server for health sciences2026 Mar 05

Esta pesquisa revela que, na dermatomiosite e em outras doenças autoimunes, os autoanticorpos não apenas circulam, mas são *internalizados* pelas células (do músculo e de outros tecidos). Uma vez dentro, esses autoanticorpos ativam programas genéticos específicos que levam a danos celulares e inflamação (como a ativação do interferon). Este achado estabelece a internalização de autoanticorros como um mecanismo fundamental e compartilhado em diversas doenças autoimunes, oferecendo a médicos e pesquisadores uma nova perspectiva para entender a patogênese e desenvolver tratamentos mais eficazes.

🇧🇷 traduzido
#4

Anti-CD19 CAR T cells for pediatric patients with treatment-refractory autoimmune diseases.

Nature medicine2026 Mar

Uma nova terapia com células CAR T anti-CD19 demonstrou resultados promissores em crianças com doenças autoimunes graves e refratárias a tratamentos convencionais, incluindo a dermatomiosite. Todos os oito pacientes neste estudo (três com dermatomiosite) apresentaram melhora clínica substancial ou resolução da doença, permitindo a descontinuação sustentada de outros imunomoduladores, com efeitos adversos geralmente leves e manejáveis. Embora estes resultados preliminares sejam encorajadores, são urgentemente necessários ensaios clínicos formais para confirmar a segurança e eficácia a longo prazo desta abordagem para doenças como a dermatomiosite.

🇧🇷 traduzido
#5

Identification of common genes and biomarkers between Dermatomyositis and rheumatoid arthritis through integrated bioinformatics.

PloS one2026

Este estudo revelou mecanismos moleculares e imunológicos comuns entre a Dermatomiosite (DM) e a Artrite Reumatoide (AR), explicando parcialmente sua comorbidade. Foram identificados os genes HCP5 e RARRES3 como potenciais biomarcadores de diagnóstico, validados em dados externos, e vias como IL-17 e alterações em células imunes (macrófagos e células T) em ambas as doenças. Estes achados abrem caminho para o desenvolvimento de ferramentas de diagnóstico mais precisas e terapias direcionadas para pacientes que sofrem de DM e AR.

🇧🇷 traduzido

Publicações recentes

Ver todas no PubMed

📚 EuropePMC8.206 artigos no totalmostrando 193

2026

Dermatomyositis with Anti-MDA5 Autoantibodies After SARS-CoV-2 mRNA Vaccination Treated with Tofacitinib: Integrating Literature Evidence and a Novel Observation.

Antibodies (Basel, Switzerland)
2026

A challenging case of facial oedemas revealing a systemic peripheral T-cell lymphoma, not otherwise specified.

European journal of dermatology : EJD
2026

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

PloS one
2026

Distinct Interferon and Intracellular Signaling Signatures in Systemic Lupus Erythematosus and Dermatomyositis by Integrative Transcriptomic and Proteomic Analysis.

ACR open rheumatology
2026

Adverse pregnancy outcomes in adult patients with idiopathic inflammatory myopathy: a systematic review and meta-analysis.

Arthritis care &amp; research
2026

Clinical Findings, Antibody Panel and Pathology of Patients with Inflammatory Myopathies in Isfahan Province, Iran.

Advanced biomedical research
2026

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

Case reports in dermatology
2026

Prevalence and Risk of Falls and Fractures in the Idiopathic Inflammatory Myopathies: A Cross-Sectional Study of 470 Patients.

Rheumatology and therapy
2026

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

Cureus
2026

Semi-quantitative analyses of muscle magnetic resonance imaging for pattern recognition in early idiopathic inflammatory myopathies.

Journal of neuromuscular diseases
2026

The role of extracorporeal membrane oxygenation in the management of rapidly progressive interstitial lung disease due to anti-melanoma differentiation-associated gene 5 antibody dermatomyositis: A case series and brief literature review.

JHLT open
2026

Inhibition of the type I interferon receptor pathway protects against muscle weakness induced by dermatomyositis serum.

Annals of the rheumatic diseases
2026

Checkerboard Hyperkeratosis With Apoptotic Keratinocytes as Features of Mechanic's Hands and Review of the Literature.

The American Journal of dermatopathology
2026

Degos-Like-Skin Lesions in the Setting of Dermatomyositis: A Case Series and Literature Review.

Indian dermatology online journal
2025

A Study of Cutaneous Manifestations in Patients During the Post-COVID Period in A Tertiary Care Center.

Journal of pharmacy &amp; bioallied sciences
2026

Diagnosis of a patient with pulmonary and skeletal cryptococcosis: A case report and literature review.

The Journal of international medical research
2026

Fatal Cardiomyopathy Secondary to Seronegative Immune-Mediated Necrotizing Myopathy: A Case Report.

The American journal of case reports
2026

Palmar Cutaneous Erythematous Macules: A Case Report of an Atypical Presentation of Chilblain Lupus Erythematosus.

Cureus
2026

"The Cancer that Carried the Chalk"-NXP2+ Paraneoplastic Dermatomyositis Unleashing Calcinosis Cutis and Peripheral Neuropathy.

The Journal of the Association of Physicians of India
2026

Elevated Serum Levels of β1,4-Galactosyltransferase 1 Have Diagnostic Value in Patients With Dermatomyositis.

International journal of rheumatic diseases
2026

Immune dysregulation and infection susceptibility in Anti-MDA5 dermatomyositis.

Seminars in arthritis and rheumatism
2026

Linear and streaky rash in a young female.

Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG
2026

Anti-PM/Scl Antibody-Positive Dermatomyositis With Rapidly Progressive Interstitial Lung Disease in a 19-Year-Old Male: Clinical Implications of a Presumptive Diagnosis Based on Line Immunoassay Positivity.

Cureus
2026

Association of diagnostic delay with disease activity and cumulative damage in Hispanic patients with idiopathic inflammatory myopathies: a cross-sectional study.

Clinical rheumatology
2026

Global landscape of juvenile dermatomyositis research: a bibliometric analysis.

Pediatric research
2026

Clinical Value of Bronchiectasis and Airspace Enlargement Depicted on Iodine Map in Patients With Interstitial Lung Disease: A Pilot Study.

Cureus
2026

Comprehensive Analysis Reveals Potential Molecular Targets in Juvenile Dermatomyositis.

Biochemistry research international
2026

Evaluating the value of lactic dehydrogenase in anti-MDA5-positive Dermatomyositis associated with interstitial lung disease.

Frontiers in medicine
2026

A case of anti-nuclear matrix protein 2 antibody-positive dermatomyositis sine dermatitis with a challenging diagnosis due to the absence of typical skin manifestations.

Modern rheumatology case reports
2026

Cooccurrence of Anterior Cruciate Ligament Rupture and Ganglion Cyst: Clinical Complexity and Ultrasound-Guided Tailored Management.

Pain practice : the official journal of World Institute of Pain
2026

Amyopathic dermatomyositis associated with anti-MDA5 antibodies and rapidly progressive interstitial pneumonia: 2 cases with different clinical evolution.

Medicina clinica
2026

Parallel course of calcinosis and cancer in a patient with anti-NXP2-positive dermatomyositis: A case report.

Modern rheumatology case reports
2026

Clinical characteristics and risk factors associated with objective flares in patients with dermatomyositis.

Seminars in arthritis and rheumatism
2026

Dermatomyositis masquerading as angioedema: a crucial differential not to overlook.

BMJ case reports
2026

Recurrent anti-TIF1γ-positive dermatomyositis coexisting with postoperative parotid lymphoepithelial carcinoma: a case report with pathogenesis analysis.

Frontiers in immunology
2026

Flagellate erythema and breast infiltration.

JAAD case reports
2026

Overfitting, sparse data bias, and variable selection in the predictive model for rapidly progressive interstitial lung disease in dermatomyositis.

Respiratory medicine
2026

Evaluating the Diagnostic Potential of Myxovirus Resistance Protein 1 (MX1) and Myxovirus Resistance Protein 2 (MX2) As Biomarkers in Idiopathic Inflammatory Myopathies.

Cureus
2026

Dermatomyositis: Prevalence, Clinical Spectrum, Diagnostic Approach, and Management Strategies.

Archives of internal medicine research
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

Rethinking rituximab in neuroimmunology: Real-world efficacy, cost-effectiveness, and long-term remission.

Journal of neuroimmunology
2026

Periorbital heliotrope erythema with edema as a potential clinical marker of paraneoplastic Dermatomyositis - a case series.

Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG
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

Spatial transcriptomics reveals mechanism of autoimmunity driven by internalized autoantibodies.

medRxiv : the preprint server for health sciences
2026

Clinical features, treatment, and outcomes of anti-PD-1/PD-L1 immune checkpoint inhibitors induced dermatomyositis.

Immunopharmacology and immunotoxicology
2026

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

ACR open rheumatology
2026

Fitbit as an activity monitor in idiopathic inflammatory myopathy: results from a real-world cohort.

Clinical and experimental rheumatology
2026

Neutrophil-to-lymphocyte ratio as a prognostic indicator in anti-MDA5 dermatomyositis with interstitial lung disease and its link to IFN-α and IL-6.

Clinical and experimental rheumatology
2026

Acquired generalised lipodystrophy as an uncommon complication of anti-Mi-2 dermatomyositis: a case report.

Clinical and experimental rheumatology
2026

Temporal Dissociation Between Clinical Remission and Microvascular Improvement Assessed by AI-Assisted Capillaroscopy in Anti-Mi-2 Dermatomyositis: Potential Therapeutic Implications.

Arthritis &amp; rheumatology (Hoboken, N.J.)
2026

A Case of Anti-SAE Antibody-Positive Dermatomyositis Coexisting With Sjögren's Disease.

The Journal of dermatology
2026

Genetic Whispers in Hyperphosphataemic: Tumor Calcinosis in a 30-year-old.

Irish medical journal
2026

Improved Detection of Myositis-Specific Autoantibodies Using Luciferase Immunoprecipitation Systems Assay: Comparison with Line Blot and Conventional Immunoprecipitation.

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

Long-term management challenges in postmyopathic dermatomyositis.

Dermatology online journal
2026

Erythematous rash and proximal muscle weakness in a 59-year-old woman: Delayed diagnosis of dermatomyositis in a patient with skin of color.

Journal of the National Medical Association
2026

Development and validation of a multi-institutional electronic juvenile idiopathic arthritis phenotype.

Seminars in arthritis and rheumatism
2026

Association between Oropharyngeal Dysphagia and Subgroups of Patients with Idiopathic Inflammatory Myopathy: A Cross-Sectional Study.

Dysphagia
2026

The efficacy of transbronchial lung cryobiopsy for chronic progressive changes after remission of anti-MDA5 antibody-positive interstitial lung disease: a report of two cases.

Respiratory investigation
2026

Anti-MDA5 monoclonal antibodies from patients with dermatomyositis - B cell characteristics and differential targeting of the helicase domains.

Journal of autoimmunity
2026

Atypical dermatomyositis with positive anti-nuclear matrix protein 2 antibodies complicated by rhabdomyolysis syndrome: a case report and literature review.

Frontiers in immunology
2026

Pretreatment Cytokine Signatures as Candidate Biomarkers for JAK Inhibitor Response in Anti-MDA5 Dermatomyositis-Related Interstitial Lung Disease: A Pilot Study.

ACR open rheumatology
2026

Elevated serum interleukin-38 levels in polymyositis and dermatomyositis: diagnostic implications and correlations with inflammatory markers.

Frontiers in immunology
2026

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

Inflammation
2026

Acute muscle injury and subcutaneous edema in a patient with anti-nuclear matrix protein-2 antibody positive dermatomyositis sine dermatitis: A Case Report.

Internal medicine (Tokyo, Japan)
2026

Complement activation and M2-like macrophage accumulation in anti-MDA5 monoclonal antibody-induced hepatic injury in mice.

Frontiers in immunology
2026

Anti-MDA5 Antibody-Positive Dermatomyositis in Emirati Patients: A Case Series Highlighting Clinical Variability.

The American journal of case reports
2026

A case study of delayed-diagnosed leprosy: advancing diagnosis through MetaPath.

International journal of infectious diseases : IJID : official publication of the International Society for Infectious Diseases
2026

How loss-of-function mutations in IFIH1 contribute to infectious and/or inflammatory disease - a systematic review.

Journal of translational autoimmunity
2026

Anti-SAE Antibody-Positive Dermatomyositis Presenting With Widespread Erythema Complicated by Rosai-Dorfman Disease.

The Journal of dermatology
2026

Prognostic value of HRCT-based risk stratification for acute/subacute progression in polymyositis/dermatomyositis-associated interstitial lung disease.

Frontiers in immunology
2026

Postpartum-Onset Dermatomyositis Presenting With Malignant Pleural Effusion: A Case Report.

Cureus
2026

Calcinosis Cutis Universalis: A Review of Therapeutic Strategies and Surgical Management.

Journal of clinical medicine
2026

Distinct anti-MDA5 antibody trajectories associated with long-term outcomes in 6-month survivors of anti-MDA5 dermatomyositis with interstitial lung disease.

Respiratory research
2026

Validation of simplified scoring systems for assessing cutaneous disease activity in dermatomyositis.

Clinical and experimental rheumatology
2026

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

Indian journal of pediatrics
2026

Detection of dermatomyositis skin rashes in skin of color: Addressing critical training gaps.

The American journal of medicine
2026

Multicentre development and validation of data-driven claims-based algorithms for identifying dermatomyositis and polymyositis in Japan.

Modern rheumatology
2026

Dermatomyositis Mimicking Statin-Induced Myopathy in A 62-Year-Old Woman.

European journal of case reports in internal medicine
2026

Integrated proteomic and transcriptomic profiles reveals the role of OAS3 in dermatomyositis pathogenesis.

Frontiers in immunology
2026

Monocyte-driven vasculopathy distinguishes dermatomyositis from CLE.

Nature reviews. Rheumatology
2026

Early detection and intervention in dermatomyositis: current evidence and clinical implications.

Clinical rheumatology
2026

Development of enzyme-linked immunosorbent assays for the detection of myositis-specific autoantibodies against signal recognition particle, nuclear matrix protein 2, and small ubiquitin-like modifier activating enzyme.

Modern rheumatology
2026

Elbow Gottron's Sign as a Predictor of Dermatomyositis With Respiratory System as the Initial Manifestation: A Retrospective Study.

The Journal of dermatology
2026

Cytomegalovirus DNAemia in patients with anti-melanoma differentiation-associated gene 5 positive dermatomyositis from southern China.

BMC infectious diseases
2026

Anti-Melanoma Differentiation-Associated Gene 5 Antibody-Positive Interstitial Lung Disease, Induced by Enfortumab Vedotin Plus Pembrolizumab for Advanced Urothelial Carcinoma.

IJU case reports
2026

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

Pediatric pulmonology
2026

Identifying cutaneous dermatomyositis disease area and severity index thresholds of disease severity in alignment with patient perspectives on quality of life: A retrospective analysis.

Journal of the American Academy of Dermatology
2026

Pulmonary alveolar proteinosis developing during tofacitinib treatment for anti-MDA5 antibody-positive dermatomyositis-associated interstitial lung disease: a case report and review of the literature.

Immunological medicine
2026

Critical illness with fatal muscle weakness and gastrointestinal perforation after COVID-19.

Modern rheumatology case reports
2026

Anti-CD19 CAR T cells for pediatric patients with treatment-refractory autoimmune diseases.

Nature medicine
2026

Proteomic profiles in inclusion body myositis and polymyositis with mitochondrial pathology.

Acta neuropathologica communications
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

Identification of common genes and biomarkers between Dermatomyositis and rheumatoid arthritis through integrated bioinformatics.

PloS one
2026

Atypical Interplay of Dermatomyositis, Metastatic Caecal Carcinoma, and Connective Tissue Disease.

Cureus
2026

[Expression of the melanoma 2-mediated pyroptosis pathway in peripheral blood mononuclear cells of patients with idiopathic inflammatory myopathies].

Beijing da xue xue bao. Yi xue ban = Journal of Peking University. Health sciences
2026

Phospho-MLKL Upregulation in Immune-Mediated Necrotizing Myopathy: Implications for Disease Pathophysiology and Diagnosis.

Neuropathology : official journal of the Japanese Society of Neuropathology
2026

Prognostic and Functional Trajectories in Idiopathic Pulmonary Fibrosis and Systemic Autoimmune Rheumatic Disease-Associated Interstitial Lung Disease: Insights From an Italian Multicenter Cohort.

The Journal of rheumatology
2026

Paraneoplastic dermatomyositis with atypical features associated with a solid pseudopapillary pancreatic neoplasm.

Clinical rheumatology
2026

Thrombotic microangiopathy in a patient with anti-signal recognition particle antibody-positive immune-mediated necrotising myopathy: a case report.

Modern rheumatology case reports
2025

Extracellular Vesicular Proteins in Plasma from Patients with Cutaneous Lupus Correlate with Disease Activity.

Current issues in molecular biology
2025

Idiopathic Inflammatory Myopathy With Normal Creatine Kinase Levels in an Elderly Patient: A Diagnostic Challenge.

Cureus
2026

Clinical Characteristics and Evolution of Interstitial Lung Disease in Subtypes of Idiopathic Inflammatory Myositis With Prevalent Lung Manifestation: A Retrospective Analysis.

ACR open rheumatology
2026

Muscle RING finger-1 facilitates skeletal muscle regeneration via regulating myoblast proliferation and differentiation.

Journal of translational medicine
2026

Hyperglycemia-A Driver of Cutaneous Severity in Dermatomyositis: A Narrative Review.

Journal of clinical medicine
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

Characteristics, treatments and outcomes of patients with dermatomyositis using real-world data.

RMD open
2025

A Diagnostic Delay: Respiratory Muscle Weakness in Dermatomyositis Masquerading as Pneumonia.

Cureus
2026

An Overview of Small-Molecule Agents for the Treatment of Dermatomyositis.

Current topics in medicinal chemistry
2026

Kikuchi-Fujimoto Disease as the Initial Presentation of Dermatomyositis.

Current rheumatology reviews
2025

Different faces of anti-melanoma differentiation-associated gene 5 antibody-positive myositis.

Reumatologia
2026

Dysregulation of YTHDF2 promotes the over expression of interferon beta in anti-MDA5 antibody-positive dermatomyositis.

Arthritis research &amp; therapy
2026

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

Rheumatology (Oxford, England)
2026

Serum interferon-λ3 as a short-term biomarker of disease control in anti-MDA5-positive dermatomyositis-associated ILD.

Scientific reports
2026

IgG from anti-MDA5⁺ CADM patients impairs NK cell function via CD16 in RP-ILD.

Translational research : the journal of laboratory and clinical medicine
2026

Intravitreal Clindamycin for Multifocal Toxoplasma Retinochoroiditis in Immunocompromised Patients: A Case Series with Aqueous Humor PCR Confirmation.

Ocular immunology and inflammation
2025

When viral infections meet the anti-MDA5 antibody-positive dermatomyositis.

Frontiers in immunology
2025

From Rhabdomyolysis to a Lymphoproliferative Disorder: A Long Diagnostic Work-Up.

Cureus
2026

Serum ferritin trajectories predict mortality risk in anti-melanoma differentiation-associated protein 5 antibody-positive dermatomyositis: a longitudinal cohort study.

The European respiratory journal
2026

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

International journal of dermatology
2026

Alterations of mucosal-associated invariant T cells in dermatomyositis.

Clinical and experimental rheumatology
2026

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

Clinical and experimental rheumatology
2026

Marked Long-Term Improvement in Lung Function in Melanoma Differentiation-Associated Protein 5 Antibody-Positive Dermatomyositis Patients: Experience of a Single-Center Longitudinal Cohort in North America.

Arthritis care &amp; research
2025

Post-COVID-19 Inflammatory Myopathy With Dermatomyositis-Mixed Connective Tissue Disease (MCTD) Overlap Features and Epiglottitis: A Case Report.

Cureus
2026

Stevens-Johnson syndrome/toxic epidermal necrolysis as the initial presentation of paraneoplastic anti-TIF1-γ dermatomyositis.

SAGE open medical case reports
2026

Dermatomyositis and Polymyositis: Are Both Ends of a Same Spectrum?

Journal of cosmetic dermatology
2026

Clinical prognosis and cancer risk assessment in autoimmune diseases with anti-TIF1γ alone or concurrent with anti-Ro52 antibodies in a Taiwanese cohort.

Clinical immunology (Orlando, Fla.)
2026

Lenabasum, a cannabinoid type 2 receptor agonist, exerts anti-inflammatory effects in dermatomyositis.

The Journal of investigative dermatology
2026

A Case of Antinuclear Antibody-Negative Dermatomyositis with Interstitial Lung Disease.

Clinical laboratory
2026

JAK Inhibition Restores Epidermal Homeostasis and Modulates Immune Responses in Dermatomyositis Skin: A Spatial Transcriptomic Pilot Study.

The Journal of dermatology
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

The clinical values of laboratory inflammatory and composite indices in predicting rapidly progressive interstitial lung disease and prognosis in anti-MDA5 dermatomyositis patients.

Clinical rheumatology
2026

The Refractory DermatoMyositis Index (ReDMI): a clinical tool to predict refractory disease in patients with dermatomyositis.

Clinical and experimental rheumatology
2026

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

Clinical and experimental rheumatology
2025

Dermatomyositis following COVID-19 Vaccination: A Case Report and Review of Vaccine-associated Autoimmune Phenomena.

Acta medica Philippina
2026

[Interferon scores in the management of rheumatic systemic diseases].

Zeitschrift fur Rheumatologie
2026

Immunotherapies in autoimmune inflammatory myopathies: Rationale and therapeutic updates.

Handbook of clinical neurology
2026

Intravenous immunoglobulin use in neurology.

Handbook of clinical neurology
2026

Semiquantitative HRCT scoring: a reliable predictor of rapidly progressive interstitial lung disease in patients with anti-MDA5 positive dermatomyositis.

Quantitative imaging in medicine and surgery
2026

Evaluating heterogeneity in MDA-5+ dermatomyositis through cluster analyses.

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

Energy-Based Devices for the Treatment of Cutaneous Lesions in Patients With Lupus Erythematosus and Dermatomyositis.

Journal of cosmetic dermatology
2026

Sera from patients with dermatomyositis and antisynthetase syndrome mediate muscle weakness, impair mitochondrial respiration and induce local cytokine production in muscle tissue.

Journal of autoimmunity
2025

Risk Factors Associated with the Development of Thrombotic Microangiopathy in Patients with Dermatomyositis.

International journal of molecular sciences
2026

Peripheral blood immunophenotypic diversity in patients with anti-MDA5+ dermatomyositis and its impact on prognosis.

Clinical and experimental rheumatology
2026

NXP-2-positive dermatomyositis presenting with pronounced unilateral lower extremity subcutaneous edema.

JAAD case reports
2026

Prolonged remission of dermatomyositis and autoantibody negativization following SARS-CoV-2 infection in an elderly patient.

Autoimmunity
2026

Validation of International Classification of Diseases Codes for Dermatologic Conditions: A Systematic Review.

JAMA dermatology
2026

Interferon-β Triggers Z-DNA Binding Protein 1-Mediated PANoptosis in T Cells of Anti-Melanoma Differentiation-Associated Protein 5 Antibody-Positive Dermatomyositis.

Immunology
2025

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

Frontiers in immunology
2025

Pregnancy outcomes in idiopathic inflammatory myopathies: a Portuguese multicentre study.

Frontiers in medicine
2026

Nailfold videocapillaroscopy in the assessment of juvenile connective tissue diseases.

Clinical and experimental rheumatology
2026

CTRP1 regulates skeletal muscle differentiation through quality control of mitochondrial dynamics and function.

Molecular therapy : the journal of the American Society of Gene Therapy
2026

Anti-MDA5 antibody-positive interstitial lung disease without prominent skin or muscle manifestations: A high-risk and potentially overlooked subgroup.

Respiratory medicine
2026

Baricitinib alleviates myositis-associated interstitial lung disease in mice by inhibiting the NETs-cGAS-STING-EMT axis.

International immunopharmacology
2025

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

Case reports in pediatrics
2025

Identifying immunostimulatory herbal supplements that may flare autoimmune skin diseases: a systematic scoping review.

Lupus science &amp; medicine
2025

Paraneoplastic dermatomyositis with Kaposi's sarcoma: A case report.

SAGE open medical case reports
2026

Genetic Association Between Polymyositis/Dermatomyositis and Epilepsy: Insights From Mendelian Randomization and Bioinformatic Analyses.

Brain and behavior
2026

Paraneoplastic Amyopathic Dermatomyositis Suspect by Nailfold Capillaroscopy in a Patient With Breast Carcinoma.

International journal of rheumatic diseases
2026

Flagellate Erythema: An Indicator of Underlying Malignancy in Dermatomyositis.

International journal of rheumatic diseases
2025

Transcriptomic signatures in different tissues of anti-MDA5 positive dermatomyositis: Uncovering genes with predictive potential for RP-ILD.

Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG
2026

Concurrent paraneoplastic dermatomyositis and Sweet syndrome.

JAAD case reports
2025

Dermatomyositis is characterized by JAK1-mediated monocyte-driven vasculopathy and inflammation.

Science translational medicine
2025

An Update on Dermatomyositis and Related Inflammatory Myopathies: Cutaneous Clues, Skeletal Muscle Involvement, and Advances in Pathogenesis and Treatment.

Muscles (Basel, Switzerland)
2025

Exhaustion of NK cells and interferon activation in anti-MDA5+ dermatomyositis are associated and determine the development of ILD.

Frontiers in immunology
2026

Macrophage activation syndrome and rapidly progressive lung disease as life-threatening manifestations of anti-MDA5 antibody disease. A case report.

Reumatismo
2025

Unraveling the genetic association between autoimmune thyroid diseases and idiopathic inflammatory myopathies in the European population.

Medicine
2025

Sit to stand and timed up and go in idiopathic inflammatory myopathies.

Scientific reports
2025

Dermatomyositis in women: a 15-year retrospective analysis of clinical patterns, malignancy risk, and long-term outcomes at a tertiary center.

Acta dermatovenerologica Alpina, Pannonica, et Adriatica
2025

Case Report: Low-Dose Tofacitinib Reverses Progressive Interstitial Lung Disease in Anti-MDA5 Dermatomyositis After Failure of Traditional Immunosuppression.

International journal of rheumatic diseases
2025

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

Pediatric pulmonology
2025

Concomitant IgA nephropathy and dermatomyositis: a case-based review.

Rheumatology international
2025

Serum Autoantibodies in Dermatomyositis and Systemic Lupus Erythematosus: A Systematic Review and Meta-Analysis.

Journal of cutaneous medicine and surgery
2026

Juvenile dermatomyositis as a rare paraneoplastic manifestation of nasopharyngeal carcinoma.

Rheumatology (Oxford, England)
2025

Critical Limb Ischaemia in a Patient With Subcutaneous Nodules: Clues to a Hidden Systemic Disease.

Cureus
2026

Real-world experience of anifrolumab in 30 patients with refractory DM: a multicentre-multidisciplinary retrospective study.

Rheumatology (Oxford, England)
2025

[Clinical characteristics, efficacy and safety of antifibrotic agents in elderly patients with connective tissue disease-associated interstitial lung disease].

Beijing da xue xue bao. Yi xue ban = Journal of Peking University. Health sciences
2025

[Rheumatic disease spectrum and immunological profile of anti-PM/Scl antibodies in idiopathic inflammatory myopathies].

Beijing da xue xue bao. Yi xue ban = Journal of Peking University. Health sciences
2025

Corrigendum to "Diagnosis of neuropsychiatry disorder in patients with anti-MDA5 antibody dermatomyositis: A case report" [Heliyon Volume 9, Issue 12, December 2023, Article e22935].

Heliyon
2025

Efficacy of anifrolumab on skin, joint and lung involvement in anti-MDA5 positive dermatomyositis: a case report.

Frontiers in immunology
2025

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

Expert opinion on biological therapy
2025

Association of dermatological manifestations with in fertility: A systematic review of literature.

JPMA. The Journal of the Pakistan Medical Association
2025

Drooling as a Red Flag: Insights From a Case Series in Severe Dermatomyositis With Literature Review.

Clinical case reports
2025

CaNO and eCO Might Be Potential Non-Invasive Biomarkers for Disease Severity and Exacerbations in Interstitial Lung Disease.

Journal of clinical medicine
2025

New-onset anti-melanoma differentiation-associated gene 5 antibody positive clinically amyopathic dermatomyositis following COVID-19: A case report.

Internal medicine (Tokyo, Japan)
2025

RF-New Treatments in Dermatomyositis: Present and Future.

Actas dermo-sifiliograficas
2026

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

Journal of paediatrics and child health
2025

Artifactual hypoglycemia caused by Raynaud's phenomenon: A case report with literature review.

Frontiers in endocrinology
2025

Unilateral Facial Swelling as the Inaugural Sign of Anti-NPX2 Dermatomyositis: A Diagnostic Challenge in Oral Medicine.

Case reports in dentistry
2025

Cardiac involvement in patients with anti-MDA5 Ab+ dermatomyositis: a cross-sectional study with cardiac magnetic resonance.

Quantitative imaging in medicine and surgery
2025

Prognostic factors and a nomogram for survival in anti-MDA5 antibody-positive dermatomyositis-associated interstitial lung disease.

BMC pulmonary medicine
2026

Muscle mitochondrial changes in antisynthetase syndrome and other idiopathic inflammatory myopathies.

Journal of neuropathology and experimental neurology
2025

Musculoskeletal Diseases: Mechanisms and Therapeutic Advances.

MedComm
2026

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

European journal of radiology
Ver todos os 8.206 no EuropePMC

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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. Dermatomyositis masquerading as angioedema: a crucial differential not to overlook.
    BMJ case reports· 2026· PMID 41781005mais citado
  3. Spatial transcriptomics reveals mechanism of autoimmunity driven by internalized autoantibodies.
    medRxiv : the preprint server for health sciences· 2026· PMID 41757163mais citado
  4. Anti-CD19 CAR T cells for pediatric patients with treatment-refractory autoimmune diseases.
    Nature medicine· 2026· PMID 41644747mais citado
  5. Identification of common genes and biomarkers between Dermatomyositis and rheumatoid arthritis through integrated bioinformatics.
    PloS one· 2026· PMID 41637423mais citado
  6. Association between IL1B rs16944 polymorphism and the risk of idiopathic inflammatory myopathies.
    Front Immunol· 2026· PMID 41993189recente
  7. Systematic literature review informing PReS/CARRA-endorsed recommendations for the treat-to-target strategy in juvenile dermatomyositis.
    J Rheumatol· 2026· PMID 41985922recente
  8. Remission rates and predictors in idiopathic inflammatory myopathy subgroups: Insights from a single-center cohort.
    J Rheumatol· 2026· PMID 41985918recente
  9. Lung transplant outcomes in myositis, systemic sclerosis and idiopathic pulmonary fibrosis: a multicenter retrospective analysis.
    Rheumatology (Oxford)· 2026· PMID 41984803recente
  10. Upadacitinib Versus Tofacitinib in Anti-MDA5-Positive Dermatomyositis With Interstitial Lung Disease: A Multi-Centre Cohort Study Emulating a Target Trial.
    Respirology· 2026· PMID 41983711recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:221(Orphanet)
  2. MONDO:0016367(MONDO)
  3. Dermatomiosite e Polimiosite(PCDT · Ministério da Saúde)
  4. GARD:6263(GARD (NIH))
  5. Busca completa no PubMed(PubMed)
  6. Artigo Wikipedia(Wikipedia)
  7. Q681160(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
Compêndio · Raras BR

Dermatomiosite

ORPHA:221 · MONDO:0016367
🇧🇷 Brasil SUS
Geral
Prevalência
1-9 / 100 000
Herança
Not applicable
CID-10
M33.1 · Outras dermatomiosites
CID-11
Ensaios
29 ativos
Início
Adult, Elderly
Prevalência
1.97 (Australia)
MedGen
UMLS
C0011633
Repurposing
2 candidatos
potassium-p-aminobenzoateglucocorticoid receptor agonist
prednisone
EuropePMC
Wikidata
Wikipedia
Papers 10a
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