Where are we going in the management of interstitial lung disease in patients with systemic sclerosis?

Autoimmun Rev. 2015 Jul;14(7):575-8. doi: 10.1016/j.autrev.2015.02.002. Epub 2015 Feb 21.


Interstitial lung disease (ILD) affects about 90% of patients with systemic sclerosis (SSc). It is associated with a restrictive lung disease in only 30% of patients and is progressive in an even lower percentage. A low forced vital capacity at presentation, an extent of lung fibrosis >20% as detected by lung high-resolution computed tomography, high serum interleukin-6 levels, anti-topoisomerase I antibody positivity and diffuse cutaneous SSc are each associated with SSc-ILD progression. However, no such association is absolute. Treating patients with a recent deterioration of lung function may allow to capture those with active disease. To date, cyclophosphamide (CYC) is the only drug found to stabilize or improve lung function in randomized clinical trials, but its small beneficial effect is short lived. Therefore, immunosuppressive maintenance therapy after CYC treatment is warranted. At present, however, the best therapeutical strategy after CYC therapy both in responders and in non-responders to CYC is still controversial. Based on a review of the literature, we suggest an approach to the management of SSc-ILD.

Keywords: Active SSc-ILD; First-line treatment; Second-line treatment.

Publication types

  • Review

MeSH terms

  • Cyclophosphamide / therapeutic use
  • Disease Progression
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Lung Diseases, Interstitial / etiology
  • Lung Diseases, Interstitial / therapy*
  • Scleroderma, Systemic / complications*
  • Scleroderma, Systemic / diagnosis
  • Stem Cell Transplantation


  • Immunosuppressive Agents
  • Cyclophosphamide