Independent Predictors of Mortality in Systemic Sclerosis-Associated Pulmonary Arterial Hypertension: A Systematic Review and Meta-Analysis

Abstract

The aim of this study is to determine the predictors of mortality in patients with systemic sclerosis-induced pulmonary arterial hypertension (SSc-PAH). This systematic review and meta-analysis were carried out according to the Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA) Statement guidelines. We searched the PubMed, EMBASE, and Web of Science databases from January 2010 to April 2023 using the following keywords: "systemic sclerosis," "pulmonary arterial hypertension," "death," and "predictors," along with medical subject headings (MeSH), to identify relevant studies. A total of eight studies with a total of 530 patients were included in the present systematic review and meta-analysis. The pooled one-year, three-year, and five-year survival was 90% (95% CI: 86-93%), 66% (95% CI: 59-72%), and 44% (95% CI: 23-65%), respectively. Factors associated with mortality in SSc-PAH included age (p-value: 0.02), male gender (p-value: 0.008), pericardial effusion (p-value: 0.003), cardiac index (p-value: 0.0001), six-minute walking distance (p-value: 0.04), pulmonary arterial pressure (PAP) (p-value: 0.01), and New York Heart Association (NYHA) classification (p-value: 0.0002). The findings of this study have important clinical implications. Assessing and managing the identified predictors, such as age, gender, pericardial effusion, PAP, cardiac index, and NYHA class, could help identify individuals at higher risk of mortality and guide treatment strategies.