Ken J. Bulpitt, MD; Philip J. Clements, MD; Peter A. Lachenbruch, PhD; Harold E. Paulus, MD; James B. Peter, MD, PhD; Mel S. Agopian, MS; Joyce Z. Singer, MD; Virginia D. Steen, MD; Daniel O. Clegg, MD; Carol M. Ziminski, MD; Graciela S. Alarcon, MD, MPH; Michael E. Luggen, MD; Richard P. Polisson, MD; Robert F. Willkens, MD; James C. Reading, PhD; H. James Williams, MD; John R. Ward, MD
To characterize the course of early scleroderma and to delineate prognostic factors present within 1 year of disease onset that might identify patients at high risk.
Inception cohort study.
Ten university-based rheumatology clinics participating in the Cooperative Systematic Studies of Rheumatic Diseases Program.
Forty-eight patients who had had scleroderma for less than 1 year.
Fifteen patients with early scleroderma who died were compared with those still living during the initial study period (1982 to 1992). Kaplan-Meier survival estimation and Cox proportional-hazards analysis were used to analyze baseline variables for their ability to predict survival duration.
Eight of 15 deaths were due to cardiac or pulmonary system failure. The estimated 5-year survival rate was 68%. Baseline factors that were the most predictive of a poor outcome included the presence of abnormal cardiopulmonary signs and abnormal urine sediment (pyuria, hematuria).
Evidence of early cardiopulmonary disease, renal disease, inflammation, or immune activation may identify a subset of patients with scleroderma who will experience rapidly progressive disease and early death.
Bulpitt KJ, Clements PJ, Lachenbruch PA, et al. Early Undifferentiated Connective Tissue Disease: III. Outcome and Prognostic Indicators in Early Scleroderma (Systemic Sclerosis). Ann Intern Med. 1993;118:602–609. doi: 10.7326/0003-4819-118-8-199304150-00005
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Published: Ann Intern Med. 1993;118(8):602-609.
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