{
  "abstract": "Background\n\nCombined 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\n\nIn 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\n\nCPFE-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\n\nCPFE-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\n\nChinese Clinical Study.org, No: ChiCTR2000032212, Registration Date: 2020-04-23; URL: http://www.chictr.org.cn/ .",
  "abstract_shown": true,
  "affiliations": [
    "Department of Pulmonary and Critical Care Medicine, the First Affiliated Hospital with Nanjing Medical University, Nanjing, Jiangsu, 210029, China.",
    "Department of Pulmonary Circulation, Shanghai Pulmonary Hospital, Tongji University School of Medicine, 507 Zhengmin Road, Shanghai, 200433, China.",
    "Department of Pathology, Shanghai Pulmonary Hospital, Tongji University School of Medicine, 507 Zhengmin Road, Shanghai, 200433, China.",
    "Department of Respiratory and Critical Care Medicine, Nanjing Drum Tower Hospital, Nanjing, Jiangsu, 210008, China.",
    "Department of Pulmonary and Critical Care Medicine, the Affiliated Wuxi People's Hospital of Nanjing Medical University, Wuxi, Jiangsu, 214023, China.",
    "Department of Ultrasound, Nanjing Drum Tower Hospital, Nanjing, Jiangsu, 210008, China.",
    "Department of Pathology, Shanghai Pulmonary Hospital, Tongji University School of Medicine, 507 Zhengmin Road, Shanghai, 200433, China. wuchunyan581@163.com.",
    "Department of Pulmonary Circulation, Shanghai Pulmonary Hospital, Tongji University School of Medicine, 507 Zhengmin Road, Shanghai, 200433, China. gongsugang@tongji.edu.cn.",
    "Department of Pulmonary Circulation, Shanghai Pulmonary Hospital, Tongji University School of Medicine, 507 Zhengmin Road, Shanghai, 200433, China. lanwang@tongji.edu.cn."
  ],
  "article_type": "research article",
  "arxiv": null,
  "author_total": 12,
  "authors": [
    {
      "affs": [
        0,
        1
      ],
      "name": "Mengyu He"
    },
    {
      "affs": [
        1
      ],
      "name": "Qinhua Zhao"
    },
    {
      "affs": [
        2
      ],
      "name": "Huikang Xie"
    },
    {
      "affs": [
        1
      ],
      "name": "Rui Zhang"
    },
    {
      "affs": [
        1
      ],
      "name": "Jinming Liu"
    },
    {
      "affs": [
        0
      ],
      "name": "Weiping Xie"
    },
    {
      "affs": [
        3
      ],
      "name": "Yanzhe Yu"
    },
    {
      "affs": [
        4
      ],
      "name": "Yan Wu"
    },
    {
      "affs": [
        5
      ],
      "name": "Jiayi Zhang"
    },
    {
      "affs": [
        6
      ],
      "name": "Chunyan Wu"
    },
    {
      "affs": [
        7
      ],
      "name": "Sugang Gong"
    },
    {
      "affs": [
        8
      ],
      "name": "Lan Wang"
    }
  ],
  "bibtex": "https://research.draconitic.com/paper/2ssadnfm2pna8.bib",
  "cited_by": 0,
  "doi": "10.1186/s12931-026-03828-7",
  "fulltext_url": "https://europepmc.org/articles/PMC13689075",
  "id": "2ssadnfm2pna8",
  "issns": [
    "1465-9921",
    "1465-993X"
  ],
  "landing_url": "https://europepmc.org/article/MED/42907855",
  "language": "eng",
  "licence": "CC-BY-NC-ND",
  "licence_label": "CC BY-NC-ND",
  "licence_source": "article",
  "licence_url": "https://creativecommons.org/licenses/by-nc-nd/4.0/",
  "metadata_licence": null,
  "native_subjects": [
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      "lcc",
      "RC705-779"
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      "openalex",
      "2740|Pulmonary Hypertension Research and Treatments"
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  "pdf_url": "https://europepmc.org/articles/PMC13689075?pdf=render",
  "pmcid": "PMC13689075",
  "pmid": "42907855",
  "published": "2026-07-01",
  "publisher": "BMC",
  "ris": "https://research.draconitic.com/paper/2ssadnfm2pna8.ris",
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    {
      "first_seen": "2026-10-30T12:19:57Z",
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  "subjects": [
    {
      "field": "medicine",
      "origin": "openalex:2740|Pulmonary Hypertension Research and Treatments",
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  ],
  "title": "Clinical, radiological, and pathological features of pulmonary hypertension associated with combined pulmonary fibrosis and emphysema.",
  "title_text": "Clinical, radiological, and pathological features of pulmonary hypertension associated with combined pulmonary fibrosis and emphysema.",
  "type": "peer-reviewed",
  "updated_at": "2026-10-30T13:46:21Z",
  "url": "https://research.draconitic.com/paper/2ssadnfm2pna8",
  "venue": "Respiratory research",
  "versions": []
}