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Virchows Archiv

, Volume 474, Issue 1, pp 97–104 | Cite as

Pleuroparenchymal fibroelastosis as a histological background of autoimmune diseases

  • Yoshiaki Kinoshita
  • Kentaro Watanabe
  • Hiroshi Ishii
  • Hisako Kushima
  • Makoto Hamasaki
  • Masaki Fujita
  • Kazuki Nabeshima
Original Article
  • 126 Downloads

Abstract

Patients with autoimmune disease–related interstitial lung disease (AID-ILD) occasionally develop radiologic pleuroparenchymal fibroelastosis (PPFE)–like lesions. However, the significance of AID as an etiology of PPFE has not been fully elucidated. The aim of this study is to verify the increase of elastic fibers in AID-ILD patients and evaluate the prevalence of histological PPFE in patients with AID-ILD. We selected cases of clinically diagnosed AID-ILD and idiopathic pulmonary fibrosis (IPF), in which an autopsy had been performed or in which the patient had undergone pneumonectomy for lung transplantation. We quantified the collagen fibers and elastic fibers in each lobe as the percentage of the non-aerated lung area (collagen fiber score and elastic fiber score, respectively) in histological specimens from a total of 73 patients (AID-ILD, n = 24; IPF, n = 49). There were no significant differences in the collagen fiber scores of the AID-ILD and IPF groups. Meanwhile, the elastic fiber scores of the AID-ILD group were significantly greater than those of the IPF group in the whole lung (17.3 ± 7.70 vs 11.6 ± 4.55), and the upper (16.6 ± 8.11 vs 11.2 ± 5.18), and lower (18.0 ± 9.68 vs 12.0 ± 5.55) lobes (all p < 0.01). Histological PPFE pattern was found in 12 of 24 AID-ILD patients (50%), and histological PPFE pattern as a dominant pattern of fibrosis was found in 2 of the 24 patients (8%). Thus, PPFE can be a manifestation of AID-ILD.

Keywords

Pleuroparenchymal fibroelastosis Elastic fibers Vasculitis Usual interstitial pneumonia Connective tissue disease 

Notes

Acknowledgements

The authors thank Ms. H. Fukagawa, Department of Pathology, Fukuoka University School of Medicine and Hospital, for her skillful assistance with the preparation and staining of the tissue samples.

Author contribution

YK contributed to the design and concept of the study, data analysis, and writing of the manuscript. KW contributed to the design and concept of the study, data analysis, and development of the manuscript. KN contributed to the data analysis and gave advice on the design and concept of the study. HI, HK, MH, and MF contributed to the collection of the data and final approval of the manuscript.

Funding

This study was partially supported by a grant from the Ministry of Health, Labor and Welfare of Japan awarded to the Study Group on Diffuse Pulmonary Disorders, Scientific Research/Research on Intractable Diseases.

Compliance with ethical standards

This study was approved by The Fukuoka University Hospital Institutional Review Board, and it approved the study protocol and waived the requirement for informed consent.

Conflict of interest

The authors declare that they have no conflicts of interest.

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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2018

Authors and Affiliations

  • Yoshiaki Kinoshita
    • 1
    • 2
  • Kentaro Watanabe
    • 1
    • 3
  • Hiroshi Ishii
    • 1
  • Hisako Kushima
    • 1
  • Makoto Hamasaki
    • 2
  • Masaki Fujita
    • 1
  • Kazuki Nabeshima
    • 2
  1. 1.Department of Respiratory MedicineFukuoka University HospitalFukuokaJapan
  2. 2.Department of PathologyFukuoka University School of Medicine and HospitalFukuokaJapan
  3. 3.General Medical Research CenterFukuoka University School of MedicineFukuokaJapan

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