Ann Rheum Dis 66:940-944 doi:10.1136/ard.2006.066068
  • Extended report

Changes in causes of death in systemic sclerosis, 1972–2002

  1. Virginia D Steen1,
  2. Thomas A Medsger2
  1. 1Georgetown University, Washington, DC, USA
  2. 2School of Medicine, University of Pittsburgh, Pittsburgh, Pennsylvania, USA
  1. Correspondence to:
    Dr V D Steen
    Georgetown University, 3800 Reservoir Road, LL Kober Cogan, Washington, DC 20007, USA; steenv{at}
  • Accepted 18 February 2007
  • Published Online First 28 February 2007


Background: Survival of scleroderma has changed since the renal crisis treatment has become possible.

Aims: To document the changes in survival and organ system causes of mortality in systemic sclerosis (SSc) over the past 25 years in patients from a single medical centre.

Methods: Consecutive patients evaluated at the University of Pittsburgh, Pittsburgh, Pennsylvania, USA between 1 January 1972 and 31 December 1996 were studied. Survival was determined in five 5-year time periods between 1972 and 1997. Causes of death included scleroderma-related (scleroderma renal crisis, pulmonary arterial hypertension, pulmonary fibrosis (PF), gastrointestinal (GI), heart and multiorgan failure) and non-scleroderma-related (cancer, atherosclerotic cardiovascular or cerebrovascular disease, infection, sudden death, other and unknown) causes.

Results: The 10-year survival improved steadily from 54% to 66% during each of the time intervals. There was a significant improvement in survival for patients during 1982–91 compared with those during 1972–81 (p<0.001), even when patients with renal crisis were excluded (p<0.005). The frequency of deaths due to renal crisis significantly decreased over the 30-year time period, from 42% to 6% of scleroderma-related deaths (p<0.001), whereas the proportion of patients with scleroderma who died of PF increased from 6% to 33% (p<0.001). The frequency of pulmonary hypertension, independent of PF, also significantly increased during this time period (p<0.05). There were no changes in scleroderma GI- and heart-related deaths, nor in any of the non-scleroderma-related causes, although patients with scleroderma were less likely to die from scleroderma-related problems in the past 15 years.

Conclusion: The change in the pattern of scleroderma-related deaths over the past 30 years demonstrates that the lung (both pulmonary hypertension and PF) is the primary cause of scleroderma-related deaths today. It is important that aggressive searches continue to develop better therapies for these severe pulmonary complications of SSc.


  • Published Online First 28 February 2007

  • Funding: This study was funded partly by a grant from the Scleroderma Foundation, Danvers, Massachusetts, USA.

  • Competing interests: None declared.