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

  • Other CTD: Inflammatory Myopathies and Sjögren's (P Basharat, Section Editor)
  • Published:
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Abstract

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.

Summary

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

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Authors and Affiliations

  1. Department of Pulmonary and Critical Care Medicine, and Multi-Organ Transplant Institute, Ochsner Clinic Foundation, New Orleans, LA, USA

    Courtney L. Shappley DO

  2. Tulane University School of Medicine, Section of Pulmonary Medicine; Section of Rheumatology, New Orleans, LA, USA

    Courtney L. Shappley DO & Lesley Ann Saketkoo MD, MPH

  3. Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, MD, USA

    Julie J. Paik MD, MHS

  4. University Medical Center Comprehensive Pulmonary Hypertension Center, New Orleans, LA, 70112, USA

    Lesley Ann Saketkoo MD, MPH

  5. New Orleans Scleroderma and Sarcoidosis Patient Care and Research Center, Tulane University School of Medicine, New Orleans, LA, 70112, USA

    Lesley Ann Saketkoo MD, MPH

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Shappley, C.L., Paik, J.J. & Saketkoo, L.A. Myositis-Related Interstitial Lung Diseases: Diagnostic Features, Treatment, and Complications. Curr Treat Options in Rheum 5, 56–83 (2019). https://doi.org/10.1007/s40674-018-0110-6

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