Scleroderma Patients Are Living Longer

WASHINGTON, July 13 -- The lifespan for patients with scleroderma has increased significantly since the 1970s, in large measure because of a decline in deaths from renal disease.

A review of data on more than 2,000 patients with scleroderma treated from 1972 through 2001 showed that 10-year survival improved from 54% to 66%, reported Virginia Steen, M.D., of Georgetown University, and Thomas Medsger, M.D., of the University of Pittsburgh.

The investigators found that survival of scleroderma patients improved in each five-year increment studied, and that deaths from renal crisis significantly decreased from 42% in 1972 to just 6% in 2001, they reported in the July issue of Annals of the Rheumatic Diseases.

However, deaths from pulmonary fibrosis increased from 6% to 33%, and there were concomitant increases in deaths from pulmonary hypertension.

The investigators looked at data on 2,105 patients in a scleroderma patient registry developed by Dr. Steen when she was a fellow at the University of Pittsburgh in the early 1980s.

They determined whether the death of each patient was related to scleroderma, and, if so, into which of six categories it should be classified:

• Scleroderma renal crisis, including deaths related to dialysis or renal transplantation,
• Pulmonary arterial hypertension,
• Pulmonary fibrosis,
• Gastrointestinal, including severe involvement of the esophagus or small bowel, resulting in malabsorption, malnutrition or hyperalimentation,
• Cardiac, including primary cardiomyopathy and arrhythmias or conduction defects not attributable to other cardiac conditions and requiring treatment,
• Multi-system organ failure.

Drs. Steen and Medsger saw that there was a steady improvement in 10-year survival for each of the time intervals, and significant improvement in survival for patients from 1982 through 1991 compared with the 1972 through 1981 period (P