Rheumatology

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Survival, causes of death, and risk factors associated with mortality in Spanish systemic sclerosis patients: results from a single university hospital
Seminars in Arthritis and Rheumatism, 09/29/09

Joven BE et al. – Ten-year survival is over 70% in Spanish SSc patients. The main causes of death are lung and cardiac involvement, and to a lesser extent, peripheral vascular disease and coexisting malignancy. Diffuse subset, proteinuria, PH, elevated erythrocyte sedimentation rate and older age at diagnosis are the main risk factors for mortality.

• Demographic, clinical, and outcome data from all SSc patients followed in rheumatology department included in database created in 1989
• ANOVA, Kruskal-Wallis, or χ 2 tests were used to identify differences among groups
• Cox proportional hazards regression analysis used to identify factors associated with mortality

• 204 patients were included; 182 (89%) were women
• Mean age at diagnosis 49 ± 17 years
• Mean follow-up was 8 y
• Over 1635 patient-y
• 36 of 44 deaths were attributable to SSc: 28 related to cardiorespiratory involvement, 4 to peripheral vascular disease, 3 to gastrointestinal, and 1 to renal involvement
• Main SSc-unrelated causes of death were malignancy (3 cases) and infections (2 cases)
• Survival rates from disease onset 85, 75, and 55% at 5, 10, and 20 years
• Poorer survival in patients with renal disease and pulmonary hypertension (PH)
• Independent prognostic factors for mortality were older age at diagnosis, diffuse skin involvement, proteinuria, PH, and elevated ESR

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