Interstitial Lung Disease and Other Pulmonary Manifestations in Connective Tissue Diseases

Mayo Clin Proc. 2019 Feb;94(2):309-325. doi: 10.1016/j.mayocp.2018.09.002. Epub 2018 Dec 14.


Lung involvement in connective tissue diseases is associated with substantial morbidity and mortality, most commonly in the form of interstitial lung disease, and can occur in any of these disorders. Patterns of interstitial lung disease in patients with connective tissue disease are similar to those seen in idiopathic interstitial pneumonias, such as idiopathic pulmonary fibrosis. It may be difficult to distinguish between the 2 ailments, particularly when interstitial lung disease presents before extrapulmonary manifestations of the underlying connective tissue disease. There are important clinical implications in achieving this distinction. Given the complexities inherent in the management of these patients, a multidisciplinary evaluation is needed to optimize the diagnostic process and management strategies. The aim of this article was to summarize an approach to diagnosis and management based on the opinion of experts on this topic.

Publication types

  • Review

MeSH terms

  • Biopsy
  • Connective Tissue Diseases / complications*
  • Connective Tissue Diseases / diagnosis
  • Global Health
  • Humans
  • Lung / diagnostic imaging*
  • Lung Diseases, Interstitial* / diagnosis
  • Lung Diseases, Interstitial* / epidemiology
  • Lung Diseases, Interstitial* / etiology
  • Morbidity / trends
  • Pulmonary Fibrosis* / diagnosis
  • Pulmonary Fibrosis* / epidemiology
  • Pulmonary Fibrosis* / etiology
  • Respiratory Function Tests
  • Survival Rate / trends
  • Tomography, X-Ray Computed