TY - JOUR T1 - Survival, Causes of Death, and Prognostic Factors in Systemic Sclerosis: Analysis of 947 Brazilian Patients JF - The Journal of Rheumatology JO - J Rheumatol SP - 1971 LP - 1978 DO - 10.3899/jrheum.111582 VL - 39 IS - 10 AU - PERCIVAL D. SAMPAIO-BARROS AU - ADRIANA B. BORTOLUZZO AU - ROBERTA G. MARANGONI AU - LUIZA F. ROCHA AU - ANA PAULA T. DEL RIO AU - ADIL M. SAMARA AU - NATALINO H. YOSHINARI AU - JOÃO FRANCISCO MARQUES-NETO Y1 - 2012/10/01 UR - http://www.jrheum.org/content/39/10/1971.abstract N2 - Objective. To analyze survival, prognostic factors, and causes of death in a large cohort of patients with systemic sclerosis (SSc). Methods. From 1991 to 2010, 947 patients with SSc were treated at 2 referral university centers in Brazil. Causes of death were considered SSc-related and non-SSc-related. Multiple logistic regression analysis was used to identify prognostic factors. Survival at 5 and 10 years was estimated using the Kaplan-Meier method. Results. One hundred sixty-eight patients died during the followup. Among the 110 deaths considered related to SSc, there was predominance of lung (48.1%) and heart (24.5%) involvement. Most of the 58 deaths not related to SSc were caused by infection, cardiovascular or cerebrovascular disease, and cancer. Male sex, modified Rodnan skin score (mRSS) > 20, osteoarticular involvement, lung involvement, and renal crisis were the main prognostic factors associated to death. Overall survival rate was 90% for 5 years and 84% for 10 years. Patients presented worse prognosis if they had diffuse SSc (85% vs 92% at 5 yrs, respectively, and 77% vs 87% at 10 yrs, compared to limited SSc), male sex (77% vs 90% at 5 yrs and 64% vs 86% at 10 yrs, compared to female sex), and mRSS > 20 (83% vs 90% at 5 yrs and 66% vs 86% at 10 yrs, compared to mRSS < 20). Conclusion. Survival was worse in male patients with diffuse SSc, and lung and heart involvement represented the main causes of death in this South American series of patients with SSc. ER -