An increased risk of pulmonary hypertension in patients with combined pulmonary fibrosis and emphysema: a meta-analysis

Abstract

Background and aim: Pulmonary hypertension (PH) is a common complication of combined pulmonary fibrosis and emphysema (CPFE). Whether the incidence of PH is increased in CPFE compared with pure pulmonary fibrosis or emphysema remains unclear. This meta-analysis aimed to evaluate the prevalence of PH in the CPFE population. Methods We searched the PubMed, Embase, Cochrane Library, and CNKI databases for relevant studies focusing on the incidence of PH in patients with CPFE and IPF or emphysema. Pooled odds ratios (ORs) and standard mean differences (SMD) with 95% confidence intervals (95% CIs) were used to evaluate the differences in the clinical characteristics and prevalence of PH between patients with CPFE, IPF, or emphysema. The survival impact of PH in patients with CPFE was assessed using hazard ratios (HRs). Results A total of 19 eligible studies were included in the meta-analysis, involving 977, 1153, and 504 patients with CPFE, IPF, and emphysema, respectively. Patients with CPFE had an increased PH risk with a higher frequency of pulmonary hypertension and higher estimated systolic pulmonary artery pressure (esPAP), compared with those with IPF (OR: 1.96; 95% CI: 1.36–2.83; P < 0.01; SMD: 0.79; 95% CI: 0.55–1.02; P < 0.01) or emphysema (OR: 2.09; 95% CI: 1.42–3.06; P < 0.01; SMD: 0.75; 95% CI: 0.55–0.95; P < 0.01). In addition, the patients with CPFE combined with PH had a poor prognosis than patients with CPFE without PH (HR: 6.16; 95% CI: 2.53–15.03; P < 0.01). Conclusions Our meta-analysis showed that patients with CPFE were associated with a significantly higher prevalence of PH compared with those with IPF or emphysema alone. The presence of PH was a poor predictor of mortality.

Pulmonary hypertension in chronic obstructive pulmonary disease: current understanding, knowledge gaps and future directions

“…Perhaps best representing the multiple pathways for pathogenesis of group 3 PH are patients with combined pulmonary fibrosis with emphysema (CPFE). A recent meta-analysis in CPFE found a higher incidence of PH in CPFE than idiopathic pulmonary fibrosis (IPF) or emphysema alone and confirmed the association of PH in this population with increased mortality [6]. In summary, these studies highlight the heterogenous mechanisms for pathogenesis and mixed severity of pulmonary vascular disease in PH-COPD and underscore the need for more specific phenotyping of PH-COPD for clinical trials and registry analyses.…”