Interstitial lung disease in scleroderma

Rheum Dis Clin North Am. 2015 May;41(2):237-48. doi: 10.1016/j.rdc.2014.12.005. Epub 2015 Feb 26.


Systemic sclerosis is a heterogeneous disease of unknown etiology with limited effective therapies. It is characterized by autoimmunity, vasculopathy, and fibrosis and is clinically manifested by multiorgan involvement. Interstitial lung disease is a common complication of systemic sclerosis and is associated with significant morbidity and mortality. The diagnosis of interstitial lung disease hinges on careful clinical evaluation and pulmonary function tests and high-resolution computed tomography. Effective therapeutic options are still limited. Several experimental therapies are currently in early-phase clinical trials and show promise.

Keywords: Diagnosis; Fibrosis; Interstitial lung disease; Pathogenesis; Systemic sclerosis; Treatment.

Publication types

  • Review

MeSH terms

  • Humans
  • Lung Diseases, Interstitial / diagnosis*
  • Lung Diseases, Interstitial / etiology
  • Lung Diseases, Interstitial / physiopathology
  • Lung Diseases, Interstitial / therapy
  • Scleroderma, Systemic / complications*