Background and aimPulmonary 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 risk of PH in patients with CPFE compared to those with IPF or COPD/emphysema.MethodsWe 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 presence and severity of PH between patients with CPFE, IPF, or emphysema. The survival impact of PH in patients with CPFE was assessed using hazard ratios (HRs).ResultsA total of 13 eligible studies were included in the meta-analysis, involving 560, 720, and 316 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: 2.66; 95% CI: 1.55-4.57;P< 0.01; SMD: 0.86; 95% CI: 0.52-1.19;P< 0.01) or emphysema (OR: 3.19; 95% CI: 1.42-7.14;P< 0.01; SMD: 0.73; 95% CI: 0.50-0.96;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).ConclusionsOur meta-analysis showed that patients with CPFE were associated with a significantly higher risk of PH compared with those with IPF or emphysema alone. The presence of PH was a poor predictor of mortality.Supplementary InformationThe online version contains supplementary material available at 10.1186/s12890-023-02425-4.