People who suffer from scleroderma (systemic sclerosis) are living significantly longer, compared with 30 years ago. Scleroderma, a rare autoimmune disorder of unknown origin, results when collagen builds up in the body's connective tissue and causes thickening of the skin. Progressively, scleroderma causes stiff joints and hardening of the internal organs and blood vessels.
Physicians who treat this rare disease of connective tissue hope the newer drugs now on the market may extend lives longer.
In the July 2007 issue of the journal Annals of the Rheumatic Diseases, a Georgetown professor studied 2,000 patients with scleroderma treated between 1972 and 2001 at the University of Pittsburgh and found that 10-year survival steadily improved over those years by 12 percent; from 54 percent to 66 percent.
The reason overall mortality has dropped is partly because a class of medications, angiotensin converting enzyme (ACE) inhibitors became available which dramatically improved the treatment of renal crisis. Renal crisis used to be almost always fatal and now it is a very treatable complication of scleroderma.
Now pulmonary arterial hypertension and pulmonary fibrosis, a condition that is traditionally the second-most frequent cause of death, is responsible for most of the mortality, but these conditions may be increasingly treatable.
While all scleroderma-related disorders are believed to affect as many as 900,000 people in the United States, only between 40,000 and 165,000 are diagnosed with systemic scleroderma. Like all scleroderma disorders, systemic scleroderma affects more women than men, and varies in severity.
The study found survival improved in each of the five-year periods, and that the frequency of death due to renal crisis significantly decreased over the 30 years, from 42 percent to 6 percent. But the proportion of patients who died from pulmonary fibrosis increased from 6 percent to 33 percent. The frequency of pulmonary hypertension also increased, but there was no change in gastrointestinal and heart-related deaths.
Use of ACE inhibitors made all the difference in saving patients from renal failure. It's important that physicians use drugs now available to treat lung disorders and researchers continue to search for new therapies.