Connective Tissue Disease Associated Interstitial Lung Disease

Immunol Allergy Clin North Am. 2023 May;43(2):229-244. doi: 10.1016/j.iac.2023.01.005. Epub 2023 Mar 3.

Abstract

Connective tissue disease associated interstitial lung disease (CTD-ILD) is a heterogenous collection of conditions with a diverse spectrum of interstitial lung disease (ILD) manifestations. Currently, clinical practice of lung-directed immunosuppression in CTD-ILD is supported by several randomized, placebo-controlled trials (RCTs) in patients with scleroderma and several observational, retrospective studies in other autoimmune conditions. However, given the harm of immunosuppression in idiopathic pulmonary fibrosis, there is an urgent need for RCTs of immunosuppression and antifibrotic agents in fibrotic CTD-ILD populations as well as the study of intervention in patients with subclinical CTD-ILD.

Keywords: Antifibrotic; Autoimmunity; Connective tissue disease; Immunosuppression; Interstitial lung disease; Subclinical interstitial lung disease.

Publication types

  • Review
  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Connective Tissue Diseases* / complications
  • Humans
  • Idiopathic Pulmonary Fibrosis* / complications
  • Idiopathic Pulmonary Fibrosis* / diagnosis
  • Idiopathic Pulmonary Fibrosis* / therapy
  • Lung
  • Lung Diseases, Interstitial* / diagnosis
  • Lung Diseases, Interstitial* / etiology
  • Lung Diseases, Interstitial* / therapy