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Keywords:

  • connective tissue disease;
  • interstitial lung disease;
  • collagen vascular disease;
  • undifferentiated connective tissue disease;
  • autoimmune-featured interstitial lung disease;
  • lung-dominant connective tissue disease

ABSTRACT

The intersection of interstitial lung disease (ILD) and connective tissue disease (CTD) is complex and commonly includes the scenario whereby ILD is identified in patients with pre-existing CTD, is the presenting manifestation of an occult CTD, or arises within the context of a suggestive form of CTD. Determining that an ILD is CTD-associated is important because this knowledge often impacts management and prognosis. Identifying occult CTD in patients with presumed idiopathic ILD can be challenging and requires a comprehensive, often multidisciplinary, evaluation. There is much uncertainty and controversy surrounding the suggestive forms of CTD-associated ILD (CTD-ILD) and prospective studies are needed to provide a better understanding of the natural history of these cohorts, how to best manage them, and to determine whether they behave similar to classifiable forms of CTD-ILD. © 2014 American College of Rheumatology.