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

Semin Arthritis Rheum. 2014 Oct;44(2):208-19. doi: 10.1016/j.semarthrit.2014.05.010. Epub 2014 May 14.

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.

Keywords: Mortality; Systemic sclerosis.

Publication types

  • Meta-Analysis
  • Review
  • Systematic Review

MeSH terms

  • Adult
  • Female
  • Humans
  • Lung Diseases / etiology
  • Lung Diseases / mortality
  • Male
  • Middle Aged
  • Scleroderma, Systemic / complications
  • Scleroderma, Systemic / mortality*
  • Survival Rate
  • Time Factors