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

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 for mortality in those with interstitial lung disease is 2.6. The average life expectancy of patients with SSc is 16-34 years less than age-matched and sex-matched population peers. Current research efforts are focused on quantifying early as well as late mortality, and modeling for predictors of death in SSc, with the ultimate goal of attenuating this risk and improving survival, as new therapies emerge.

SUMMARY: Studies have consistently shown a substantially increased mortality in SSc, predominantly due to cardio-pulmonary complications. A better understanding of risk factors for mortality holds the promise of improving outcomes in this devastating multiorgan autoimmune disease.