title: Mortality and survival in systemic sclerosis: systematic review and meta-analysis.
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.
authors.affiliation: Autoimmune Diseases Unit, Bellvitge University Hospital, Barcelona, Spain. Electronic address: mrubio@bellvitgehospital.cat.
authors.forename: Manuel
authors.initials: M
authors.lastname: Rubio-Rivas
authors.affiliation: Autoimmune Diseases Unit, Bellvitge University Hospital, Barcelona, Spain.
authors.forename: Cristina
authors.initials: C
authors.lastname: Royo
authors.affiliation: Vall d'Hebron University Hospital, Barcelona, Spain.
authors.forename: Carmen Pilar
authors.initials: CP
authors.lastname: Simeón
authors.affiliation: Autoimmune Diseases Unit, Bellvitge University Hospital, Barcelona, Spain.
authors.forename: Xavier
authors.initials: X
authors.lastname: Corbella
authors.affiliation: Vall d'Hebron University Hospital, Barcelona, Spain.
authors.forename: Vicent
authors.initials: V
authors.lastname: Fonollosa
mesh_terms: D000328:Adult; D005260:Female; D006801:Humans; D008171:Lung Diseases / Q000209:etiology / Q000401:mortality; D008297:Male; D008875:Middle Aged; D012595:Scleroderma, Systemic / Q000150:complications / Q000401:mortality*; D015996:Survival Rate; D013997:Time Factors
keywords: Mortality; Systemic sclerosis