Survival, Causes of Death, and Prognostic Factors in Systemic Sclerosis: Analysis of 947 Brazilian Patients

• Published in: Journal of rheumatology Vol. 39; no. 10; pp. 1971 - 1978
• Main Authors: SAMPAIO-BARROS, Percival D, BORTOLUZZO, Adriana B, MARANGONI, Roberta G, ROCHA, Luiza F, DEL RIO, Ana Paula T, SAMARA, Adil M, YOSHINARI, Natalino H, MARQUES-NETO, Joao Francisco
• Format: Journal Article
• Language: English
• Published: Toronto, ON Journal of Rheumatology Publishing 01-10-2012
• Subjects: Adult; Age Factors; Aged; Aged, 80 and over; Biological and medical sciences; Brazil - epidemiology; Cause of Death; Disease Progression; Diseases of the osteoarticular system; Female; Humans; Male; Medical sciences; Middle Aged; Prognosis; Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis; Scleroderma, Systemic - mortality; Sex Factors; Survival Rate; Brazil; Heart; Human; Immunopathology; Connective tissue disease; Skin disease; CAUSES OF DEATH; Prognosis; SYSTEMIC SCLEROSIS; Lung; SURVIVAL ANALYSIS; Rheumatology; HEART INVOLVEMENT; Autoimmune disease; Survival; Systemic disease; Scleroderma; LUNG INVOLVEMENT
• ISSN: 0315-162X; 1499-2752
• DOI: 10.3899/jrheum.111582

To analyze survival, prognostic factors, and causes of death in a large cohort of patients with systemic sclerosis (SSc).
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.
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).
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.