Mortality in systemic sclerosis: lessons learned from population-based and observational cohort studies

Abstract

PURPOSE OF REVIEW: Systemic sclerosis (SSc) has a case-based mortality that is one of the highest among the rheumatic diseases. This article is an appraisal of current knowledge regarding survival, causes of death and risk factors for reduced life-expectancy in systemic sclerosis (SSc). RECENT FINDINGS: Recent systematic reviews of cohorts studies published worldwide have revealed a pooled standardized mortality ratio in SSc of 3.5, and reiterated the importance of heart-lung involvement as a major cause of death in this disease. Indeed, the pooled hazard ratio (HR) of mortality in SSc patients with pulmonary arterial hypertension (PAH) compared with those without is 3.5, while the pooled HR..