Myositis-Related Interstitial Lung Diseases: Diagnostic Features, Treatment, and Complications

  • Courtney L. Shappley
  • Julie J. Paik
  • Lesley Ann SaketkooEmail author
Other CTD: Inflammatory Myopathies and Sjögren's (P Basharat, Section Editor)
Part of the following topical collections:
  1. Topical Collection on Other CTD: Inflammatory Myopathies and Sjogren's


Purpose of the review

Idiopathic inflammatory myopathies (IIMs) are a group of rare, heterogeneous connective tissue diseases marked by skeletal muscle inflammation. The four main forms of IIMs include dermatomyositis (DM), polymyositis (PM), inclusion body myositis (IBM), and immune-mediated necrotizing myopathy (IMNM). Anti-synthetase syndrome (ASyS) is a very important classification of the IIM phenotypical spectrum and can be associated with rapidly progressive pulmonary disease. Each form of myositis, except inclusion body myositis, can be associated with multisystem organ involvement including pulmonary, cardiac, arthritis, and skin.

Recent findings

The lung in DM, PM and ASyS is the most common extra-muscular organ in IIM with a spectrum ranging from mild to fatal interstitial lung disease (ILD) with or without muscle involvement. Given that there is a subset of myositis patients with rapidly progressive ILD, it is important that rheumatologists in particular assess for lung involvement in all patients with myositis. In recent years, serologic testing for myositis autoantibodies has been more readily available allowing clinicians to order these tests commercially. The evaluation of a patient with myositis not only includes clinical phenotyping but also close observation to tendency for and rapidity of lung progression as well as serologic phenotyping with autoantibodies which can guide the treatment plan and prognosis of patients with ILD.


Herein, essential aspects of recognition, diagnosis, monitoring of progression, scope of treatment and IIM-ILD related complications are discussed.


Idiopathic inflammatory myopathies Myosits Anti-synthetase Interstitial lung disease Restrictive lung disease Polymyositis Dermatomyositis Immune-mediated necrotizing myopathy 


Compliance with Ethical Standards

Conflict of Interest

Courtney Shappley, Julie J. Paik, and Lesley Ann Saketkoo each declare no potential conflicts of interest.

Human and Animal Rights and Informed Consent

This article does not contain any studies with human or animal subjects performed by any of the authors.


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Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  • Courtney L. Shappley
    • 1
    • 2
  • Julie J. Paik
    • 3
  • Lesley Ann Saketkoo
    • 2
    • 4
    • 5
    Email author
  1. 1.Department of Pulmonary and Critical Care Medicine, and Multi-Organ Transplant InstituteOchsner Clinic FoundationNew OrleansUSA
  2. 2.Tulane University School of Medicine, Section of Pulmonary Medicine; Section of RheumatologyNew OrleansUSA
  3. 3.Division of RheumatologyJohns Hopkins University School of MedicineBaltimoreUSA
  4. 4.University Medical Center Comprehensive Pulmonary Hypertension CenterNew OrleansUSA
  5. 5.New Orleans Scleroderma and Sarcoidosis Patient Care and Research CenterTulane University School of MedicineNew OrleansUSA

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