Current approach to connective tissue disease-associated interstitial lung disease

Curr Opin Pulm Med. 2014 Sep;20(5):449-56. doi: 10.1097/MCP.0000000000000081.


Purpose of review: To review current approaches to the complex intersection of interstitial lung disease (ILD) with the spectrum of connective tissue disease (CTD).

Recent findings: There is a growing appreciation that the approach to CTD-associated ILD (CTD-ILD) can be enhanced by a multidisciplinary evaluation that often incorporates the rheumatologist. Determining that ILD is associated with an established CTD requires the exclusion of alternative causes and thorough assessments of the clinical features of both the CTD and ILD. The detection of occult CTD in patients with presumed 'idiopathic' disease requires careful attention to the demographic profile, historical clues, subtle physical examination findings, specific autoantibody positivity, radiologic, and histopathologic features. A comprehensive treatment program for CTD-ILD should address comorbid conditions and consider implementation of adjunctive therapeutic strategies. Pharmacologic intervention for CTD-ILD is reserved for those with progressive, clinically significant disease and typically involves use of immunosuppressive therapies.

Summary: A multidisciplinary approach can be helpful for CTD-ILD. Further research and controlled trials are needed to determine how to best manage the diverse spectrum of CTD-ILD.

Publication types

  • Review

MeSH terms

  • Autoantibodies / immunology
  • Connective Tissue Diseases / drug therapy
  • Connective Tissue Diseases / etiology*
  • Diagnosis, Differential
  • Humans
  • Lung Diseases, Interstitial / complications*


  • Autoantibodies