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Miopatias inflamatórias idiopáticas

  • Overview
  • Theory
  • Diagnosis
  • Management
  • Follow up
  • Resources
Last reviewed: 17 Aug 2025
Last updated: 16 Aug 2024

Summary

Definition

History and exam

Key diagnostic factors

  • dificuldade com tarefas motoras
  • fraqueza muscular
  • atrofia muscular
  • rash heliotrópico com edema na pálpebra
  • pápulas de Gottron
Full details

Other diagnostic factors

  • quedas frequentes
  • fadiga e mal-estar generalizado
  • perda de peso
  • sinais de insuficiência cardíaca e/ou infarto do miocárdio, arritmias, dispneia, ruídos adventícios, palpitações, síncope
  • febre leve
  • disfagia
  • mialgia
  • artralgia e/ou articulações edemaciadas
  • rash facial
  • rash eritematoso
  • alterações na dobra ungueal
  • fraqueza muscular facial
  • calcinose cutânea
  • neoplasia maligna
  • sinais sistêmicos de doença autoimune
  • Fenômeno de Raynaud
Full details

Risk factors

  • idade >40 anos
  • predisposição genética
  • sexo feminino e/ou etnia negra (polimiosite e dermatomiosite)
  • sexo masculino e/ou etnia branca (miosite de corpos de inclusão)
  • uso de estatinas
  • tabagismo
  • exposição à alta intensidade da radiação ultravioleta (UV) global
  • infecções
  • vacinação
  • uso de determinados medicamentos
  • outros fatores ambientais
Full details

Diagnostic investigations

1st investigations to order

  • creatina quinase
  • eletromiografia
  • biópsia muscular
  • autoanticorpos específicos contra miosite e associados
  • ressonância nuclear magnética
  • aldolase
  • lactato desidrogenase
  • alanina aminotransferase
  • aspartato aminotransferase (AST)
  • mioglobina
Full details

Investigations to consider

  • velocidade de hemossedimentação
  • proteína C-reativa
  • fator antinuclear
  • creatinina sérica
  • tomografia computadorizada de alta resolução dos pulmões
  • teste de função pulmonar
  • capacidade de difusão
  • ferritina sérica
  • troponinas
  • Eletrocardiograma
  • ecocardiografia
  • ressonância nuclear magnética cardíaca
  • tomografia por emissão de pósitrons com fluordesoxiglucose (FDG-PET)/tomografia computadorizada
  • ultrassonografia muscular
Full details

Treatment algorithm

ACUTE

miopatias inflamatórias idiopáticas graves: apresentação inicial

miopatias inflamatórias idiopáticas não graves: apresentação inicial

ONGOING

remissão alcançada: qualquer gravidade

Contributors

Authors

Hector Chinoy, PhD, FRCP, BMBS, MSc, BMedSci

Professor of Rheumatology and Neuromuscular Disease

National Institute for Health Research Manchester Biomedical Research Centre

Manchester University NHS Foundation Trust

The University of Manchester

Manchester

UK

Disclosures

HC has received grant support from Eli Lilly and UCB; consulting fees from Novartis, Eli Lilly, Orphazyme, AstraZeneca, Pfizer, Kezar Life Science; speaker for UCB and Biogen. He is also an author of several references cited in this topic.

James Lilleker, PhD, MBChB

Consultant Neurologist and Honorary Senior Lecturer

Muscle Disease Unit

Salford Royal Hospital Manchester

Centre for Clinical Neurosciences

Salford

Centre for Musculoskeletal Research

Division of Musculoskeletal and Dermatological Sciences

University of Manchester

Manchester

UK

Disclosures

JL has been reimbursed by Roche for consultancy, received sponsored travel and accommodation conference support from Roche, and is on the speakers’ bureau for Sanofi, and is an author of several references cited in this topic.

Choon Guan Chua, MBBS, MRCP (UK), DAvMed (UK), FRCP

Consultant Rheumatologist

Department of Rheumatology, Allergy, and Immunology

Tan Tock Seng Hospital

Singapore

Disclosures

CGC declares that he has no competing interests.

Acknowledgements

Dr Hector Chinoy and Dr James Lilleker would like to gratefully acknowledge Dr Nizar Souayah, the previous contributor to this topic.

Disclosures

NS declares that he has no competing interests.

Рецензенты

Hani Kushlaf, MD

Associate Professor of Neurology & Pathology

Director of Neuromuscular Research

University of Cincinnati

College of Medicine

Cincinnati

OH

Раскрытие информации

HK has served as a consultant on advisory boards for Alexion AstraZeneca Rare Disease, Argenx, Catalyst, Sanofi, Takeda, Immunovant, and UCB. HS has served for the speaker bureau of Sanofi.

Peer reviewer acknowledgements

BMJ Best Practice topics are updated on a rolling basis in line with developments in evidence and guidance. The peer reviewers listed here have reviewed the content at least once during the history of the topic.

Disclosures

Peer reviewer affiliations and disclosures pertain to the time of the review.

Список литературы

Our in-house evidence and editorial teams collaborate with international expert contributors and peer reviewers to ensure that we provide access to the most clinically relevant information possible.

Основные статьи

Lundberg IE, Fujimoto M, Vencovsky J, et al. Idiopathic inflammatory myopathies. Nat Rev Dis Primers. 2021 Dec 2;7(1):86. Аннотация

Schmidt J. Current classification and management of inflammatory myopathies. J Neuromuscul Dis. 2018;5(2):109-29.Полный текст  Аннотация

Oldroyd A, Lilleker J, Chinoy H. Idiopathic inflammatory myopathies: a guide to subtypes, diagnostic approach and treatment. Clin Med (Lond). 2017 Jul;17(4):322-8.Полный текст  Аннотация

Oldroyd AGS, Lilleker JB, Amin T, et al. British Society for Rheumatology guideline on management of paediatric, adolescent and adult patients with idiopathic inflammatory myopathy. Rheumatology (Oxford). 2022 May 5;61(5):1760-8.Полный текст  Аннотация

Статьи, указанные как источники

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