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Original research
Prognostic significance of radiological pleuroparenchymal fibroelastosis in Mycobacterium avium complex lung disease: a multicentre retrospective cohort study
  1. Yuya Aono1,
  2. Hironao Hozumi1,
  3. Masato Kono2,
  4. Dai Hashimoto2,
  5. Hidenori Nakamura2,
  6. Koshi Yokomura2,
  7. Shiro Imokawa3,
  8. Masahiro Shirai4,
  9. Daisuke Akahori1,
  10. Yusuke Inoue1,
  11. Kazutaka Mori1,
  12. Masato Karayama1,
  13. Yuzo Suzuki1,
  14. Kazuki Furuhashi5,
  15. Noriyuki Enomoto1,
  16. Tomoyuki Fujisawa1,
  17. Yutaro Nakamura1,
  18. Naoki Inui6,
  19. Takafumi Suda1
  1. 1Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Hamamatsu, Shizuoka, Japan
  2. 2Respiratory Medicine, Seirei Hamamatsu General Hospital, Hamamatsu, Shizuoka, Japan
  3. 3Respiratory Medicine, Iwata City Hospital, Iwata, Shizuoka, Japan
  4. 4Respiratory Medicine, National Hospital Organisation Tenryu Hospital, Hamamatsu, Shizuoka, Japan
  5. 5Clinical Laboratories, Hamamatsu University School of Medicine, Hamamatsu, Shizuoka, Japan
  6. 6Clinical Pharmacology and Therapeutics, Hamamatsu University School of Medicine, Hamamatsu, Shizuoka, Japan
  1. Correspondence to Dr Hironao Hozumi, Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Hamamatsu 431-3192, Shizuoka, Japan; hozumi{at}


Background Mycobacterium avium complex (MAC) causes chronic respiratory infectious diseases with diverse clinical features and prognoses. Pleuroparenchymal fibroelastosis (PPFE) is a rare disease characterised by pleural fibrosis with subjacent intra-alveolar fibrosis and alveolar septal elastosis, with unique chest high-resolution CT (HRCT) features (radiological PPFE). An association between recurrent respiratory infections and PPFE formation has been hypothesised; however, the clinical significance of PPFE in MAC lung disease remains unclear.

Methods This retrospective, multicentre study investigated the prevalence of radiological PPFE in patients with MAC lung disease and its association with clinical features and outcomes. Radiological PPFE was diagnosed on the basis of HRCT findings. Prognostic factors were identified using Cox proportional hazards and Fine-Gray models.

Results Of 850 consecutive patients with definite MAC lung disease, 101 (11.9%) exhibited radiological PPFE. Patients with radiological PPFE had unique characteristics, such as lower body mass index, lower survival rate (5-year cumulative survival rate, 63.1% vs 91.7%; p<0.001) and a higher incidence of respiratory-related death (5-year cumulative incidence, 31.1% vs 3.6%; p<0.001), than those without radiological PPFE. In the multivariable analysis, the presence of radiological PPFE was independently associated with all-cause mortality (adjusted HR, 4.78; 95% CI, 2.87 to 7.95; p<0.001) and respiratory-related death (adjusted HR, 3.88; 95% CI, 2.14 to 7.01; p<0.001).

Interpretation This large-scale study demonstrated that in patients with MAC lung disease, radiological PPFE was common, a phenotype associated with unique clinical features and poor prognosis, particularly respiratory-related death. The specific management of this subgroup should be established.

  • Interstitial Fibrosis
  • Rare lung diseases
  • Respiratory Infection

Data availability statement

Data are available upon reasonable request.

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Data availability statement

Data are available upon reasonable request.

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  • Contributors YA and HH: conception and design; data collection, analysis and interpretation; manuscript writing and final approval of the manuscript. MKono, DA, YI, KM, MKarayama, YS and KF: conception and design as well as data collection and analysis. DH, HN, KY, SI, MS, NE, TF, YN and NI: data collection and analysis, and supervision. TS: conception and design, manuscript writing and administrative support. All authors reviewed and confirmed the final manuscript. HH is the guarantor of the article.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.

  • Supplemental material This content has been supplied by the author(s). It has not been vetted by BMJ Publishing Group Limited (BMJ) and may not have been peer-reviewed. Any opinions or recommendations discussed are solely those of the author(s) and are not endorsed by BMJ. BMJ disclaims all liability and responsibility arising from any reliance placed on the content. Where the content includes any translated material, BMJ does not warrant the accuracy and reliability of the translations (including but not limited to local regulations, clinical guidelines, terminology, drug names and drug dosages), and is not responsible for any error and/or omissions arising from translation and adaptation or otherwise.