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Early Undifferentiated Connective Tissue Disease: III. Outcome and Prognostic Indicators in Early Scleroderma (Systemic Sclerosis)

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; and John R. Ward, MD
[+] Article and Author Information

From the Cooperative Systematic Studies of the Rheumatic Diseases Program, Coordinating Center, The University of Utah, Salt Lake City, Utah. Requests for Reprints: Ken J. Bulpitt, MD, UCLA Division of Rheumatology, 1000 Veteran Avenue, 32-47, Los Angeles, CA 90024-1670. Acknowledgments: The authors thank Kevin Nechodom, data manager for the Cooperative Systematic Studies of the Rheumatic Diseases Program database at the University of Utah, Salt Lake City, Utah, for assistance with data acquisition. Grant Support: In part by grant AR3-6834-05 from the National Institute of Health. The Cooperative Systematic Studies of Rheumatic Diseases Program study of early undifferentiated connective tissue disease was supported through grant 1-AM6-2228 from the National Institutes of Health.


Copyright 2004 by the American College of Physicians


Ann Intern Med. 1993;118(8):602-609. doi:10.7326/0003-4819-118-8-199304150-00005
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Objective: 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.

Design: Inception cohort study.

Setting: Ten university-based rheumatology clinics participating in the Cooperative Systematic Studies of Rheumatic Diseases Program.

Patients: Forty-eight patients who had had scleroderma for less than 1 year.

Measurements: 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.

Results: 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).

Conclusion: 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.

Figures

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Figure 1.
Cumulative survival probabilities: Kaplan-Meier survival curve with Greenwood confidence limits from onset of symptoms for the 48 early scleroderma patients.

Number of patients at risk listed below curve at yearly intervals.

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Figure 2.
Cumulative survival probabilities (Kaplan-Meier) for subgroups of early scleroderma patients.PP

A) Patients with baseline cardiopulmonary abnormalities on examination compared with those without abnormalities ( = 0.01); B) patients with abnormal urine sediment compared with those with normal sediment ( = 0.002). Number of patients at risk listed below curves at yearly intervals.

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Figure 3.
Cumulative survival probabilities (Kaplan-Meier) for subgroups of early scleroderma patients.P P

A) Patients with serum soluble interleukin-2 receptor (s-IL2-R) levels 1200 U/mL compared with those with <1200 U/mL ( = 0.0006); B) patients with Westergren erythrocyte sedimentation rates (ESR) 39 mm/hr compared with those with ESR <39 mm/hr ( = 0.005). Number of patients at risk listed below curves at yearly intervals.

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