Clinical, radiological, and pathological features of pulmonary hypertension associated with combined pulmonary fibrosis and emphysema.
9 affiliations
- 1Department of Pulmonary and Critical Care Medicine, the First Affiliated Hospital with Nanjing Medical University, Nanjing, Jiangsu, 210029, China.
- 2Department of Pulmonary Circulation, Shanghai Pulmonary Hospital, Tongji University School of Medicine, 507 Zhengmin Road, Shanghai, 200433, China.
- 3Department of Pathology, Shanghai Pulmonary Hospital, Tongji University School of Medicine, 507 Zhengmin Road, Shanghai, 200433, China.
- 4Department of Respiratory and Critical Care Medicine, Nanjing Drum Tower Hospital, Nanjing, Jiangsu, 210008, China.
- 5Department of Pulmonary and Critical Care Medicine, the Affiliated Wuxi People's Hospital of Nanjing Medical University, Wuxi, Jiangsu, 214023, China.
- 6Department of Ultrasound, Nanjing Drum Tower Hospital, Nanjing, Jiangsu, 210008, China.
- 7Department of Pathology, Shanghai Pulmonary Hospital, Tongji University School of Medicine, 507 Zhengmin Road, Shanghai, 200433, China. [email protected].
- 8Department of Pulmonary Circulation, Shanghai Pulmonary Hospital, Tongji University School of Medicine, 507 Zhengmin Road, Shanghai, 200433, China. [email protected].
- 9Department of Pulmonary Circulation, Shanghai Pulmonary Hospital, Tongji University School of Medicine, 507 Zhengmin Road, Shanghai, 200433, China. [email protected].
Abstract
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/ .
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BibTeX
@article{he2026clinical,
title = {{Clinical, radiological, and pathological features of pulmonary hypertension associated with combined pulmonary fibrosis and emphysema.}},
author = {Mengyu He and Qinhua Zhao and Huikang Xie and Rui Zhang and Jinming Liu and Weiping Xie and Yanzhe Yu and Yan Wu and Jiayi Zhang and Chunyan Wu and Sugang Gong and Lan Wang},
journal = {Respiratory research},
year = {2026},
month = jul,
doi = {10.1186/s12931-026-03828-7},
url = {https://europepmc.org/article/MED/42907855},
note = {CC BY-NC-ND},
}
RIS
TY - JOUR
TI - Clinical, radiological, and pathological features of pulmonary hypertension associated with combined pulmonary fibrosis and emphysema.
AU - Mengyu He
AU - Qinhua Zhao
AU - Huikang Xie
AU - Rui Zhang
AU - Jinming Liu
AU - Weiping Xie
AU - Yanzhe Yu
AU - Yan Wu
AU - Jiayi Zhang
AU - Chunyan Wu
AU - Sugang Gong
AU - Lan Wang
PY - 2026
DA - 2026/07/01
JO - Respiratory research
DO - 10.1186/s12931-026-03828-7
UR - https://europepmc.org/article/MED/42907855
AB - 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/ .
SN - 1465-9921
SN - 1465-993X
ER -