Survival, causes of death, and prognostic factors in systemic sclerosis: analysis of 947 Brazilian patients.
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).


CONCLUSIONS
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.
Division of Rheumatology, Faculdade de Medicina, Universidade de São Paulo, São Paulo, Brazil. pdsampaiobarros@uol.com.br
Percival D
PD
Sampaio-Barros
Adriana B
AB
Bortoluzzo
Roberta G
RG
Marangoni
Luiza F
LF
Rocha
Ana Paula T
AP
Del Rio
Adil M
AM
Samara
Natalino H
NH
Yoshinari
João Francisco
JF
Marques-Neto
D000328:Adult; D000367:Age Factors; D000368:Aged; D000369:Aged, 80 and over; D001938:Brazil / Q000453:epidemiology; D002423:Cause of Death; D018450:Disease Progression; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012595:Scleroderma, Systemic / Q000401:mortality*; D012737:Sex Factors; D015996:Survival Rate