Interstitial lung disease in systemic sclerosis

Semin Respir Crit Care Med. 2014 Apr;35(2):213-21. doi: 10.1055/s-0034-1371541. Epub 2014 Mar 25.


Despite many unanswered questions regarding the pathogenesis of interstitial lung disease in systemic sclerosis (SSc-ILD) and the lack of accurate epidemiological risk factors, there have been major advances in the identification and prognostic evaluation of SSc-ILD. The evaluation of disease severity is a multidisciplinary exercise, requiring the integration of pulmonary function tests, high-resolution computed tomography data, and symptomatic severity and these factors all need to be considered in the detection of disease progression. Except in a minority of patients with reversible inflammatory disease, the primary goal of treatment is the prevention of disease progression. Current treatment regimens are centered on immunosuppressive therapy with controlled treatment data largely confined to the use of cyclophosphamide. The results of two controlled trials indicate that cyclophosphamide therapy is appropriate in SSc-ILD patients with extensive fibrotic lung involvement. There is a need to broaden therapeutic approaches with the exploration of rituximab (based on recent pilot data) and antifibrotic agents, shown to have treatment effects in other fibrotic interstitial lung diseases. However, it is also important to avoid the overtreatment of SSc-ILD patients with limited nonprogressive lung involvement. In that setting, an initial policy of nonintervention but meticulous observation ("masterful inactivity with cat-like observation") is often warranted.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Antibodies, Monoclonal, Murine-Derived / therapeutic use
  • Cyclophosphamide / therapeutic use
  • Disease Progression
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Lung Diseases, Interstitial / diagnosis
  • Lung Diseases, Interstitial / etiology*
  • Lung Diseases, Interstitial / physiopathology
  • Prognosis
  • Respiratory Function Tests
  • Rituximab
  • Scleroderma, Systemic / complications*
  • Scleroderma, Systemic / physiopathology
  • Severity of Illness Index
  • Tomography, X-Ray Computed


  • Antibodies, Monoclonal, Murine-Derived
  • Immunosuppressive Agents
  • Rituximab
  • Cyclophosphamide