Increased risk of mortality in systemic sclerosis-associated pulmonary hypertension: a systemic review and meta-analysis

Abstract

Background Pulmonary hypertension (PH) is a frequent complication of systemic sclerosis (SSc) and is currently one of the primary causes of death in patients with this disease. We conducted a systematic review and meta-analysis to assess the association between PH and mortality in patients with SSc to verify trends in mortality in patients with SSc-associated PH. Methods We searched the PubMed and Embase databases for published studies on SSc-associated PH from inception to May 2021. All cohort studies in which mortality and/or survival for SSc-associated PH were reported were included in the analysis. The outcome parameters were pooled and analyzed using a random-effects model via generic inverse-variance weighting in conventional and cumulative meta-analysis. Results The literature search identified 1161 citations, and the full texts of 54 studies were examined. Sixteen articles, with a total of 7857 patients with SSc and 1140 patients with SSc-associated PH, were included in the meta-analysis. Patients with SSc-associated PH had a higher pooled risk of mortality than patients with SSc without PH (risk ratio = 3.12; 95% confidence interval: [2.44, 3.98]). Conclusions This meta-analysis revealed a higher mortality in patients with SSc-associated PH. PH was a significant predictor of death in patients with SSc. Thus, early diagnosis and treatment of PH are important in patients with SSc.

Secular Trends in Systemic Sclerosis Mortality in the United States from 1981 to 2020

“…In the United States, SSc- PAH mortality stayed steady from 2003 to 2008, then declined by 3% annually from 2008 to 2016 [ 23 ]. Furthermore, a meta-analysis revealed that SSc-PAH was significantly associated with a greater pooled risk of mortality than SSc without PAH (RR 3.12, 95% Cl 2.44–3.98) [ 24 ]. Eighteen studies comprising a total of 12,829 individuals reported the following causes of death: 19.7% cardiac disease, 16.8% ILD, 13.1% pulmonary hypertension, and 13.8% renal disease [ 25 ].…”

A signal recognition particle-related joint model of LASSO regression, SVM-RFE and artificial neural network for the diagnosis of systemic sclerosis-associated pulmonary hypertension

“…In the advanced stage, the pathological process of SSc is dominated by fibrosis and vasculopathy, which can manifest as lung fibrosis, cardiac fibrosis, pulmonary hypertension (PH), and even scleroderma renal crisis ( Asano, 2020 ). Among the major complications of SSc, PH significantly impacts the mortality of SSc patients ( Xiong et al, 2022 ). Some studies have shown that the 3-year survival rate for patients with systemic sclerosis-associated pulmonary hypertension (SSc-PH) is between 31% and 52%, while the 5-year survival rate is <50% ( Foocharoen et al, 2011 ; Humbert et al, 2011 ; Lefèvre et al, 2013 ).…”