Clinical, radiological, and pathological features of pulmonary hypertension associated with combined pulmonary fibrosis and emphysema.

BACKGROUND

Combined pulmonary fibrosis and emphysema-associated pulmonary hypertension (CPFE-PH) has emerged as a distinct third category of PH, yet its detailed clinical-hemodynamic profile, pathological features, and comparative outcomes remain poorly defined. This study aims to characterize the phenotype of CPFE-PH and compare it with chronic obstructive pulmonary disease-related PH (COPD-PH) and idiopathic interstitial pneumonia-related PH (IIP-PH).

METHODS

In this multicenter cohort study, we analyzed 113 patients with CPFE-PH, 277 with COPD-PH, and 78 with IIP-PH, all diagnosed by right heart catheterization. Clinical, physiological, imaging, pathological, treatment response, and survival data were statistically compared across groups.

RESULTS

CPFE-PH patients were older, predominantly male smokers, and exhibited preserved ventilatory function but severely reduced diffusing capacity (DLCO 31.6% predicted, IQR 24.8-37.8). They had the most severe hemodynamic compromise (mean pulmonary arterial pressure 41 mmHg, pulmonary vascular resistance 7.4 Wood units), a high prevalence of mediastinal lymphadenopathy (81.4%), and universal capillary hemangiomatosis-like remodeling, a pathological feature absent in COPD-PH and rarely observed in IIP-PH. Pulmonary arterial hypertension-targeted therapy failed to improve functional or biomarker outcomes, and transplant-free survival was shortest in CPFE-PH (median 32 months) compared with COPD-PH and IIP-PH (P < 0.05).

CONCLUSIONS

CPFE-PH appears to represent a severe phenotype within Group 3 PH, in which prominent capillary remodeling, including hemangiomatosis-like changes, may contribute to its clinical, radiological, and functional manifestations, distinguish it from other Group 3 PH types.

CLINICAL TRIAL: Chinese Clinical Study.org, No: ChiCTR2000032212, Registration Date: 2020-04-23; URL: http://www.chictr.org.cn/ .