{
  "abstract": "Background and Objective: Combined pulmonary fibrosis and emphysema (CPFE) is characterized by coexisting upper‐zone emphysema and lower‐zone fibrosis, but standardized diagnostic criteria and clinical outcomes remain poorly defined. This study aimed to evaluate survival, acute exacerbation incidence, and disease progression in CPFE patients using international classification criteria with visual assessment of emphysema extent.\n\nMethods: This multicentre prospective cohort study at 29 Japanese hospitals enrolled 1016 patients with idiopathic interstitial pneumonias (IIPs) or chronic obstructive pulmonary disease (COPD) between 2013 and 2016. CPFE was defined as ≥ 5% emphysema determined by visual assessment of CT scans for IIP patients, according to international classification criteria. Patients were followed for 5 years.\n\nResults: Among 528 IIP patients, 92 (17.4%) had CPFE. CPFE patients showed a significantly worse 5‐year survival compared with non‐CPFE IIP patients (51.4% vs. 63.1%, p = 0.026) and COPD patients (51.4% vs. 84.0%, p < 0.001). After adjustment for covariates including IIP subtype, CPFE remained independently associated with increased mortality among IIP patients (HR of 1.85 [95% CI, 1.27–2.69], p < 0.001). CPFE patients also had a significantly higher acute exacerbation rate than did non‐CPFE IIP patients (p < 0.001). The prognostic impact of CPFE was most pronounced in patients with idiopathic pulmonary fibrosis (IPF), with CPFE/IPF patients showing the worst outcomes.\n\nConclusion: Visual assessment with a ≥ 5% emphysema threshold effectively identified CPFE patients with a distinct clinical phenotype characterized by poor survival and an increased acute exacerbation incidence. These findings validate international classification criteria and emphasize the need for enhanced surveillance and tailored management strategies for this high‐risk population.",
  "abstract_shown": true,
  "affiliations": [
    "Department of Respiratory Medicine Graduate School of Medical Sciences, Kyushu University Fukuoka Japan",
    "Clinical Research Support Center Kyushu Fukuoka Japan",
    "Kirigaoka Tsuda Hospital Kitakyushu Japan",
    "Department of Respiratory Diseases NHO Fukuokahigashi Medical Center Koga Japan",
    "Department of Respiratory Medicine Japan Community Health Care Organization Kurume General Hospital Kurume Japan",
    "Department of Respiratory Medicine Fukuoka University Chikushi Hospital Chikushino Japan",
    "Department of Respiratory Medicine School of Medicine, University of Occupational and Environmental Health, Japan Kitakyushu Japan",
    "Department of Respiratory Medicine Wakamatsu Hospital of the University of Occupational and Environmental Health, Japan Kitakyushu Japan",
    "Division of Respirology, Neurology, and Rheumatology, Department of Internal Medicine Kurume University School of Medicine Kurume Japan",
    "Department of Respiratory Medicine NHO Fukuoka National Hospital Fukuoka Japan",
    "Department of Respiratory Diseases NHO Omuta National Hospital Omuta Japan",
    "Department of Respiratory Medicine Fukuoka University School of Medicine Fukuoka Japan",
    "Department of Respiratory Medicine Hamanomachi Hospital Fukuoka Japan",
    "Division of Respiratory Medicine Aso Iizuka Hospital Iizuka Japan",
    "Department of Respiratory Medicine Kitakyushu Municipal Medical Center Kitakyushu Japan",
    "Department of Respiratory Medicine Japanese Red Cross Fukuoka Hospital Fukuoka Japan",
    "Department of Respiratory Medicine Steel Memorial Yawata Hospital Kitakyushu Japan",
    "Department of Respiratory Medicine Japan Community Health Care Organization Kyushu Hospital Kitakyushu Japan",
    "Department of Respiratory Medicine Harasanshin Hospital Fukuoka Japan",
    "Kitakyushu City Hospital Organization Kitakyushu Japan"
  ],
  "article_type": "research article",
  "arxiv": null,
  "author_total": 24,
  "authors": [
    {
      "affs": [
        0
      ],
      "name": "Kazuya Tsubouchi",
      "orcid": "0000-0003-0145-8745"
    },
    {
      "affs": [
        0
      ],
      "name": "Yasuaki Kotetsu"
    },
    {
      "affs": [
        0
      ],
      "name": "Tomotsugu Takano"
    },
    {
      "affs": [
        0
      ],
      "name": "Hiroaki Ogata"
    },
    {
      "affs": [
        1
      ],
      "name": "Fumiaki Kiyomi"
    },
    {
      "affs": [
        2
      ],
      "name": "Katsuyuki Ichiki"
    },
    {
      "affs": [
        3
      ],
      "name": "Shohei Takata"
    },
    {
      "affs": [
        4
      ],
      "name": "Yasuhiko Kitasato"
    },
    {
      "affs": [
        5
      ],
      "name": "Hiroshi Ishii"
    },
    {
      "affs": [
        6
      ],
      "name": "Kazuhiro Yatera"
    },
    {
      "affs": [
        7
      ],
      "name": "Kei Yamasaki"
    },
    {
      "affs": [
        8
      ],
      "name": "Masaki Okamoto"
    },
    {
      "affs": [
        9
      ],
      "name": "Makoto Yoshida"
    },
    {
      "affs": [
        10
      ],
      "name": "Masayuki Kawasaki"
    },
    {
      "affs": [
        11
      ],
      "name": "Masaki Fujita",
      "orcid": "0000-0003-1209-1272"
    },
    {
      "affs": [
        12
      ],
      "name": "Tomohiro Ogawa"
    },
    {
      "affs": [
        13
      ],
      "name": "Kazunori Tobino"
    },
    {
      "affs": [
        14
      ],
      "name": "Eiji Harada"
    },
    {
      "affs": [
        15
      ],
      "name": "Noriaki Nakagaki"
    },
    {
      "affs": [
        16
      ],
      "name": "Masashi Komori"
    },
    {
      "affs": [
        17
      ],
      "name": "Taishi Harada",
      "orcid": "0000-0002-5145-9036"
    },
    {
      "affs": [
        18
      ],
      "name": "Hiroshi Wataya"
    },
    {
      "affs": [
        19
      ],
      "name": "Yoichi Nakanishi"
    },
    {
      "affs": [
        0
      ],
      "name": "Isamu Okamoto"
    }
  ],
  "bibtex": "https://research.draconitic.com/paper/304r3q09a76t5.bib",
  "cited_by": 1,
  "doi": "10.1002/resp.70285",
  "fulltext_url": "https://pmc.ncbi.nlm.nih.gov/articles/PMC13728530/",
  "id": "304r3q09a76t5",
  "issns": [
    "1323-7799",
    "1440-1843"
  ],
  "landing_url": "https://doi.org/10.1002/resp.70285",
  "language": "en",
  "licence": "CC-BY-4.0",
  "licence_label": "CC BY 4.0",
  "licence_source": "article",
  "licence_url": "https://creativecommons.org/licenses/by/4.0/",
  "metadata_licence": null,
  "native_subjects": [
    [
      "heading",
      "COPD"
    ],
    [
      "heading",
      "Original Article"
    ],
    [
      "openalex",
      "2740|Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis"
    ]
  ],
  "openalex": "W7167699715",
  "pdf_url": null,
  "pmcid": "PMC13728530",
  "pmid": "42417564",
  "published": "2026-07-08",
  "publisher": null,
  "ris": "https://research.draconitic.com/paper/304r3q09a76t5.ris",
  "sources": [
    {
      "first_seen": "2026-11-07T12:21:29Z",
      "native_id": "PMC13728530",
      "source": "pmc",
      "url": "https://doi.org/10.1002/resp.70285"
    }
  ],
  "subjects": [
    {
      "field": "medicine",
      "origin": "openalex:2740|Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis",
      "subfield": "general"
    }
  ],
  "title": "Clinical Outcomes and Acute Exacerbation Incidence in Combined Pulmonary Fibrosis and Emphysema",
  "title_text": "Clinical Outcomes and Acute Exacerbation Incidence in Combined Pulmonary Fibrosis and Emphysema",
  "type": "peer-reviewed",
  "updated_at": "2026-11-07T12:21:29Z",
  "url": "https://research.draconitic.com/paper/304r3q09a76t5",
  "venue": "Respirology (Carlton, Vic.)",
  "versions": []
}