Evidence-based management of rapidly progressing systemic sclerosis

Best Pract Res Clin Rheumatol. 2010 Jun;24(3):387-400. doi: 10.1016/j.berh.2009.12.002.


Systemic sclerosis has the highest case-specific mortality of any of the auto-immune rheumatic diseases, as well as causing major morbidity. It is a major clinical challenge and one that has previously provoked substantial nihilism due to the limited therapeutic options available and the perceived lack of evidence for clinical effectiveness of those treatments that are currently in use. However, this situation is changing; there are emerging data supporting efficacy for some treatment approaches for this patient group together with a growing number of exciting potential novel approaches to treatment that are moving into the clinical arena. Some of the recent clinical trials are reviewed and discussed in detail.

Publication types

  • Review

MeSH terms

  • Clinical Trials as Topic
  • Disease Progression*
  • Evidence-Based Medicine
  • Fibrosis
  • Humans
  • Hypertension, Pulmonary / etiology
  • Hypertension, Pulmonary / pathology
  • Lung Diseases / etiology
  • Lung Diseases / pathology
  • Lung Diseases, Interstitial / etiology
  • Lung Diseases, Interstitial / parasitology
  • Pulmonary Artery / pathology
  • Pulmonary Artery / physiopathology
  • Scleroderma, Diffuse / complications
  • Scleroderma, Diffuse / diagnosis
  • Scleroderma, Diffuse / therapy*
  • Scleroderma, Limited / complications
  • Scleroderma, Limited / diagnosis
  • Scleroderma, Limited / therapy*
  • Skin Diseases / etiology
  • Skin Diseases / pathology