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Updated: Jul 16, 2026
04:44
Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Changes in causes of death in systemic sclerosis, 1972-2002
Virginia D Steen1, Thomas A Medsger
1Georgetown University, Washington, DC 20007, USA. steenv@georgetown.edu
Annals of the Rheumatic Diseases
|March 3, 2007
Summary
Survival in systemic sclerosis (SSc) has improved, but lung complications like pulmonary fibrosis and hypertension are now the leading causes of death, necessitating new therapies.
Area of Science:
- Rheumatology
- Internal Medicine
- Pulmonology
Background:
- Survival rates for scleroderma have improved due to advancements in treating scleroderma renal crisis.
- Systemic sclerosis (SSc) management has evolved, impacting patient outcomes over recent decades.
Purpose of the Study:
- To analyze changes in survival rates and causes of mortality in systemic sclerosis (SSc) patients over 25 years.
- To document shifts in organ system mortality patterns within a single SSc cohort.
Main Methods:
- A retrospective study of consecutive SSc patients evaluated between 1972 and 1996.
- Survival and causes of death (scleroderma-related and non-scleroderma-related) were analyzed across five 5-year intervals.
Main Results:
- 10-year survival improved from 54% to 66%; significant gains noted between 1982-1991.
- Deaths from scleroderma renal crisis decreased significantly (42% to 6%), while pulmonary fibrosis deaths increased (6% to 33%).
- Pulmonary hypertension frequency rose, and lung-related deaths became the primary cause of SSc mortality.
Conclusions:
- Pulmonary complications, including pulmonary hypertension and fibrosis, are now the main drivers of mortality in SSc.
- The study highlights the urgent need for enhanced therapeutic strategies targeting severe pulmonary manifestations of SSc.
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