Overall and sex- and disease subtype-specific mortality in patients with systemic sclerosis : An updated...

To assess the overall and sex- and disease subtype-specific standardized mortality ratios (SMRs) in patients with systemic sclerosis (SSc).

We surveyed studies examining overall and sex- and disease subtype-specific SMRs in patients with SSc compared with the general population using MEDLINE, EMBASE, and Cochrane databases and manual searches. A meta-analysis of the overall and sex- and disease subtype-specific SMRs in patients with SSc was then performed.

Overall, 22 reports including 13,214 patients with SSc, with 4218 deaths, met the inclusion criteria. Compared to that in the general population, overall SMR was increased in patients with SSc (SMR 2.823, 95% confidence interval [CI] 2.219-3.591, p < 0.001). Stratification by region showed a significant increase in SMR in European, North American, Asian, and Oceanian populations. Sex-specific meta-analysis revealed a significant increase in overall SMR in women and men (SMR 2.929, 95% CI 2.504-3.427, p < 0.001; SMR 3.523, 95% CI 2.933-4.232, p < 0.001), with no difference in the SMR between men and women. Disease subtype-specific meta-analysis revealed a significant increase in SMR in limited cutaneous SSc (lcSSc) and diffuse cutaneous SSc (dcSSc), however, the SMR was higher in dcSSc (SMR 4.865, 95% CI 3.863-6.127, p < 0.001) than in lcSSc (SMR 2.020, 95% CI 1.570-2.599, p < 0.001).

We demonstrate that patients with SSc have 2.82-fold higher mortality rates than the general population and that the overall SMR does not depend on sex, region, and disease subtype, however, mortality is significantly higher in dcSSc than in lcSSc.