@article {SAMPAIO-BARROS1971, author = {PERCIVAL D. SAMPAIO-BARROS and ADRIANA B. BORTOLUZZO and ROBERTA G. MARANGONI and LUIZA F. ROCHA and ANA PAULA T. DEL RIO and ADIL M. SAMARA and NATALINO H. YOSHINARI and JO{\~A}O FRANCISCO MARQUES-NETO}, title = {Survival, Causes of Death, and Prognostic Factors in Systemic Sclerosis: Analysis of 947 Brazilian Patients}, volume = {39}, number = {10}, pages = {1971--1978}, year = {2012}, doi = {10.3899/jrheum.111582}, publisher = {The Journal of Rheumatology}, abstract = {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.}, issn = {0315-162X}, URL = {https://www.jrheum.org/content/39/10/1971}, eprint = {https://www.jrheum.org/content/39/10/1971.full.pdf}, journal = {The Journal of Rheumatology} }