RT Journal Article
SR Electronic
T1 Survival, Causes of Death, and Prognostic Factors in Systemic Sclerosis: Analysis of 947 Brazilian Patients
JF The Journal of Rheumatology
JO J Rheumatol
FD The Journal of Rheumatology
SP 1971
OP 1978
DO 10.3899/jrheum.111582
VO 39
IS 10
A1 PERCIVAL D. SAMPAIO-BARROS
A1 ADRIANA B. BORTOLUZZO
A1 ROBERTA G. MARANGONI
A1 LUIZA F. ROCHA
A1 ANA PAULA T. DEL RIO
A1 ADIL M. SAMARA
A1 NATALINO H. YOSHINARI
A1 JOÃO FRANCISCO MARQUES-NETO
YR 2012
UL http://www.jrheum.org/content/39/10/1971.abstract
AB 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.