Mortality and survival in systemic sclerosis: Systematic review and meta-analysis

• Published in: Seminars in arthritis and rheumatism 2014-10, Vol.44 (2), p.208-219
• Main Authors: Rubio-Rivas, Manuel, MD, Royo, Cristina, MD, Simeón, Carmen Pilar, MD, PhD, Corbella, Xavier, MD, PhD, Fonollosa, Vicent, MD, PhD
• Format: Article
• Language: English
• Subjects: Adult; Female; Humans; Lung Diseases - etiology; Lung Diseases - mortality; Male; Middle Aged; Mortality; Rheumatology; Scleroderma, Systemic - complications; Scleroderma, Systemic - mortality; Survival Rate; Systemic sclerosis; Time Factors
• ISSN: 0049-0172; 1532-866X
• DOI: 10.1016/j.semarthrit.2014.05.010

Abstract Objective To determine the mortality, survival, and causes of death in patients with systemic sclerosis (SSc) through a meta-analysis of the observational studies published up to 2013. Methods We performed a systematic review and meta-analysis of the observational studies in patients with SSc and mortality data from entire cohorts published in MEDLINE and SCOPUS up to July 2013. Results A total of 17 studies were included in the mortality meta-analysis from 1964 to 2005 (mid-cohort years), with data from 9239 patients. The overall SMR was 2.72 (95% CI: 1.93–3.83). A total of 43 studies have been included in the survival meta-analysis, reporting data from 13,529 patients. Cumulative survival from onset (first Raynaud׳s symptom) has been estimated at 87.6% at 5 years and 74.2% at 10 years, from onset (non-Raynaud׳s first symptom) 84.1% at 5 years and 75.5% at 10 years, and from diagnosis 74.9% at 5 years and 62.5% at 10 years. Pulmonary involvement represented the main cause of death. Conclusions SSc presents a larger mortality than general population (SMR = 2.72). Cumulative survival from diagnosis has been estimated at 74.9% at 5 years and 62.5% at 10 years. Pulmonary involvement represented the main cause of death.