Current Rheumatology Reports

, 15:359 | Cite as

Interstitial Lung Disease in Inflammatory Myopathies: Clinical Phenotypes and Prognosis

  • Patrick D. W. KielyEmail author
  • Felix Chua
Part of the following topical collections:
  1. Topical Collection on Inflammatory Muscle Disease


Interstitial lung disease (ILD) is a very important complication of the idiopathic inflammatory myositides (IIM), with a prevalence of approximately 40 %. Characteristic HRCT changes, most commonly NSIP, together with a restrictive ventilatory defect and an associated decline in DLco support the presence of ILD. The strongest risk factors are the presence of the anti-amino-acyl-tRNA synthetases (ARS) and anti-MDA-5 antibodies, but a raised index of suspicion for ILD should also apply to IIM patients of black ethnicity. Overall, the prognosis of ILD in IIM is good; between 50 and 66 % of cases have a stable disease course over a substantial period of time. The remaining proportion will show signs of worsening lung disease within 12 months of diagnosis. Whereas ARS antibodies and black ethnicity have no influence on ILD prognosis, detection of the anti-MDA-5 antibody carries a poor ILD outcome, in which hyperferritinaemia appears to be an important diagnostic and prognostic feature.


Interstitial lung disease Idiopathic inflammatory myositis Inflammatory myopathy Polymyositis Dermatomyositis Clinically amyopathic dermatomyositis Anti-synthetase syndrome Anti-MDA-5 antibody KL-6 Calprotectin BAFF Ferritin Phenotypes Prognosis 


Compliance with Ethics Guidelines

Conflict of Interest

Patrick D.W. Kiely declares that he has no conflict of interest.

Felix Chua declares that he has no conflict 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.


Papers of particular interest, published recently, have been highlighted as: • Of importance

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Copyright information

© Springer Science+Business Media New York 2013

Authors and Affiliations

  1. 1.Department of RheumatologySt George’s Healthcare NHS TrustLondonUK
  2. 2.Department of Respiratory MedicineSt George’s Healthcare NHS TrustLondonUK

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