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Idiopathic inflammatory myopathies

  • Overview
  • Theory
  • Diagnosis
  • Management
  • Follow up
  • Resources
Last reviewed: 19 Aug 2025
Last updated: 16 Aug 2024

Summary

定义

病史和体格检查

关键诊断因素

  • difficulty with motor tasks
  • muscle weakness
  • muscle atrophy
  • heliotrope rash with eyelid edema
  • Gottron papules
完整详情

其他诊断因素

  • frequent falls
  • fatigue and generalized malaise
  • weight loss
  • signs of heart failure and/or myocardial infarction, arrhythmias, shortness of breath, abnormal breath sounds, palpitations, syncope
  • mild fever
  • dysphagia
  • myalgia
  • arthralgia and/or swollen joint
  • facial rash
  • erythematous rash
  • nail fold changes
  • facial muscle weakness
  • skin calcinosis
  • malignancy
  • systemic signs of autoimmune disease
  • Raynaud phenomenon
完整详情

危险因素

  • age >40 years
  • genetic predisposition
  • female sex and/or black ethnicity (polymyositis and dermatomyositis)
  • male sex and/or white ethnicity (inclusion body myositis)
  • use of statins
  • tobacco smoking
  • exposure to high intensity of global UV radiation
  • infections
  • vaccination
  • use of certain drugs
  • other environmental factors
完整详情

诊断性检查

首要检查

  • creatine kinase
  • electromyograph
  • muscle biopsy
  • myositis-specific and associated autoantibodies
  • magnetic resonance imaging
  • aldolase
  • lactate dehydrogenase
  • alanine aminotransferase
  • aspartate aminotransferase
  • myoglobin
完整详情

需考虑的检查

  • erythrocyte sedimentation rate
  • C-reactive protein
  • antinuclear antibodies
  • serum creatinine
  • high resolution computed tomography of the lungs
  • pulmonary function test
  • diffusion capacity
  • serum ferritin
  • troponins
  • electrocardiography
  • echocardiography
  • cardiac magnetic resonance imaging
  • fluorodeoxyglucose positron emission tomography (FDG-PET)/computed tomography
  • muscle ultrasound scan
完整详情

治疗流程

急症处理

severe idiopathic inflammatory myopathies: initial presentation

nonsevere idiopathic inflammatory myopathies: initial presentation

持续性治疗

remission achieved: any severity

撰稿人

作者

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

利益声明

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

利益声明

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

利益声明

CGC declares that he has no competing interests.

鸣谢

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

利益声明

NS declares that he has no competing interests.

同行评议者

​Hani Kushlaf, MD

Associate Professor of Neurology & Pathology

Director of Neuromuscular Research

University of Cincinnati

College of Medicine

Cincinnati

OH

利益声明

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.

参考文献

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.

关键文献

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

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

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.全文  摘要

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.全文  摘要

参考文献

A full list of sources referenced in this topic is available to users with access to all of BMJ Best Practice.

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