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Peer-reviewedCC BY 4.0Respirology (Carlton, Vic.)

Clinical Outcomes and Acute Exacerbation Incidence in Combined Pulmonary Fibrosis and Emphysema

  • Kazuya Tsubouchi1
  • Yasuaki Kotetsu1
  • Tomotsugu Takano1
  • Hiroaki Ogata1
  • Fumiaki Kiyomi2
  • Katsuyuki Ichiki3
  • Shohei Takata4
  • Yasuhiko Kitasato5
  • Hiroshi Ishii6
  • Kazuhiro Yatera7
  • Kei Yamasaki8
  • Masaki Okamoto9
12 more authors
  • Makoto Yoshida10
  • Masayuki Kawasaki11
  • Masaki Fujita12
  • Tomohiro Ogawa13
  • Kazunori Tobino14
  • Eiji Harada15
  • Noriaki Nakagaki16
  • Masashi Komori17
  • Taishi Harada18
  • Hiroshi Wataya19
  • Yoichi Nakanishi20
  • Isamu Okamoto1
20 affiliations
  1. 1Department of Respiratory Medicine Graduate School of Medical Sciences, Kyushu University Fukuoka Japan
  2. 2Clinical Research Support Center Kyushu Fukuoka Japan
  3. 3Kirigaoka Tsuda Hospital Kitakyushu Japan
  4. 4Department of Respiratory Diseases NHO Fukuokahigashi Medical Center Koga Japan
  5. 5Department of Respiratory Medicine Japan Community Health Care Organization Kurume General Hospital Kurume Japan
  6. 6Department of Respiratory Medicine Fukuoka University Chikushi Hospital Chikushino Japan
  7. 7Department of Respiratory Medicine School of Medicine, University of Occupational and Environmental Health, Japan Kitakyushu Japan
  8. 8Department of Respiratory Medicine Wakamatsu Hospital of the University of Occupational and Environmental Health, Japan Kitakyushu Japan
  9. 9Division of Respirology, Neurology, and Rheumatology, Department of Internal Medicine Kurume University School of Medicine Kurume Japan
  10. 10Department of Respiratory Medicine NHO Fukuoka National Hospital Fukuoka Japan
  11. 11Department of Respiratory Diseases NHO Omuta National Hospital Omuta Japan
  12. 12Department of Respiratory Medicine Fukuoka University School of Medicine Fukuoka Japan
  13. 13Department of Respiratory Medicine Hamanomachi Hospital Fukuoka Japan
  14. 14Division of Respiratory Medicine Aso Iizuka Hospital Iizuka Japan
  15. 15Department of Respiratory Medicine Kitakyushu Municipal Medical Center Kitakyushu Japan
  16. 16Department of Respiratory Medicine Japanese Red Cross Fukuoka Hospital Fukuoka Japan
  17. 17Department of Respiratory Medicine Steel Memorial Yawata Hospital Kitakyushu Japan
  18. 18Department of Respiratory Medicine Japan Community Health Care Organization Kyushu Hospital Kitakyushu Japan
  19. 19Department of Respiratory Medicine Harasanshin Hospital Fukuoka Japan
  20. 20Kitakyushu City Hospital Organization Kitakyushu Japan
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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.

Methods: 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.

Results: 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.

Conclusion: 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.

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Cite

BibTeXRIS
BibTeX
@article{tsubouchi2026clinical,
  title = {{Clinical Outcomes and Acute Exacerbation Incidence in Combined Pulmonary Fibrosis and Emphysema}},
  author = {Kazuya Tsubouchi and Yasuaki Kotetsu and Tomotsugu Takano and Hiroaki Ogata and Fumiaki Kiyomi and Katsuyuki Ichiki and Shohei Takata and Yasuhiko Kitasato and Hiroshi Ishii and Kazuhiro Yatera and Kei Yamasaki and Masaki Okamoto and Makoto Yoshida and Masayuki Kawasaki and Masaki Fujita and Tomohiro Ogawa and Kazunori Tobino and Eiji Harada and Noriaki Nakagaki and Masashi Komori and Taishi Harada and Hiroshi Wataya and Yoichi Nakanishi and Isamu Okamoto},
  journal = {Respirology (Carlton, Vic.)},
  year = {2026},
  month = jul,
  doi = {10.1002/resp.70285},
  url = {https://doi.org/10.1002/resp.70285},
  note = {CC BY 4.0},
}
RIS
TY  - JOUR
TI  - Clinical Outcomes and Acute Exacerbation Incidence in Combined Pulmonary Fibrosis and Emphysema
AU  - Kazuya Tsubouchi
AU  - Yasuaki Kotetsu
AU  - Tomotsugu Takano
AU  - Hiroaki Ogata
AU  - Fumiaki Kiyomi
AU  - Katsuyuki Ichiki
AU  - Shohei Takata
AU  - Yasuhiko Kitasato
AU  - Hiroshi Ishii
AU  - Kazuhiro Yatera
AU  - Kei Yamasaki
AU  - Masaki Okamoto
AU  - Makoto Yoshida
AU  - Masayuki Kawasaki
AU  - Masaki Fujita
AU  - Tomohiro Ogawa
AU  - Kazunori Tobino
AU  - Eiji Harada
AU  - Noriaki Nakagaki
AU  - Masashi Komori
AU  - Taishi Harada
AU  - Hiroshi Wataya
AU  - Yoichi Nakanishi
AU  - Isamu Okamoto
PY  - 2026
DA  - 2026/07/08
JO  - Respirology (Carlton, Vic.)
DO  - 10.1002/resp.70285
UR  - https://doi.org/10.1002/resp.70285
AB  - 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.  Methods: 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.  Results: 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.  Conclusion: 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.
SN  - 1323-7799
SN  - 1440-1843
ER  -