Systemic sclerosis (SSc) is a disease characterized by immunologic abnormalities, microvascular involvement and tissue fibrosis. In previous studies, 10-year survival rates ranged from 50–84%. However, there are concerns that these studies, using prevalent cohorts, are underestimating mortality.
“While the prognosis of many rheumatic diseases has improved with the availability of more effective and targeted therapies, mortality in SSc remains unacceptably high,” says Mandana Nikpour, MD, PhD, from the Departments of Rheumatology and Medicine at St. Vincent’s Hospital and the University of Melbourne, Australia. “Our goal was to accurately quantify the huge burden of mortality in scleroderma, highlighting the urgent need for new, effective therapies.”
International, Multi-Center Study
The researchers studied nearly 4,400 subjects over a median follow-up of 3.0 years (interquartile range 1.0 to 5.1 years). Information was obtained from the Australian Scleroderma Cohort Study, the Canadian Scleroderma Research Group, and the Madrid University Hospital 12 de Octubre Scleroderma Cohort Study. The Madrid group is a single-center cohort and the others are multi-center.1