Case Report
Copyright ©The Author(s) 2020. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Sep 26, 2020; 8(18): 4186-4192
Published online Sep 26, 2020. doi: 10.12998/wjcc.v8.i18.4186
Unilateral pleuroparenchymal fibroelastosis as a rare form of idiopathic interstitial pneumonia: A case report
Jae-Ha Lee, Hang-Jea Jang, Jin-Han Park, Hyun-Kuk Kim, Sunggun Lee, Ji-Yeon Kim, Seong-Ho Kim
Jae-Ha Lee, Hang-Jea Jang, Jin-Han Park, Hyun-Kuk Kim, Sunggun Lee, Seong-Ho Kim, Department of Internal Medicine, Haeundae Paik Hospital, Inje University College of Medicine, Busan 48108, South Korea
Ji-Yeon Kim, Department of Pathology, Haeundae Paik Hospital, Inje University College of Medicine, Busan 48108, South Korea
Author contributions: Lee JH, the patient’s physician, reviewed the literature and was in charge of consultation with a multidisciplinary team conference at The Kanagawa Cardiovascular and Respiratory Center; Jang HJ, Kim HK and Lee S contributed to manuscript drafting; Park JH performed the transbronchial lung biopsy, and Kim JY performed the histopathological examination; Kim SH conducted the final editing and preparation of the paper for approval; all authors have read and approved the final manuscript.
Informed consent statement: Informed written consent was obtained from the patient for publication of this report and any accompanying images.
Conflict-of-interest statement: The authors declare that they have no conflict of interest.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Corresponding author: Seong-Ho Kim, PhD, Professor, Department of Internal Medicine, Haeundae Paik Hospital, Inje University College of Medicine, Haeundae-gu, Jwa-dong 1435, Busan 48108, South Korea. junjan@paik.ac.kr
Received: April 25, 2020
Peer-review started: April 25, 2020
First decision: May 15, 2020
Revised: May 15, 2020
Accepted: August 20, 2020
Article in press: August 20, 2020
Published online: September 26, 2020
Abstract
BACKGROUND

Pleuroparenchymal fibroelastosis (PPFE) is a rare idiopathic interstitial pneumonia characterized by predominantly upper lobe involvement with pleural fibrosis and subjacent parenchymal fibrosis. Recently, there have been increasing reports of PPFE, and PPFE might coexist with other interstitial lung diseases in the lower lobe and upper lobe. However, cases of unilateral PPFE are scarce.

CASE SUMMARY

A 75-year-old Korean male presented to our hospital with chronic dry cough and exertional dyspnea. The patient’s symptoms started 6 mo previously and had been gradually worsening. At the time of presentation, he felt dyspnea when walking at his own pace. Radiologic findings suggested PPFE, but the lesion was localized in the upper lobe of the right lung. After multidisciplinary discussion, a transbronchial lung biopsy in the right upper lobe revealed collapsed alveoli with parenchymal fibroelastosis, and elastic van Gieson staining demonstrated septal elastosis with intra-alveolar collagenosis, which met the histopathologic criteria of definite PPFE. After multidisciplinary discussion in an experienced interstitial lung disease center, we confirmed the diagnosis of unilateral PPFE. Furthermore, we confirmed the progression of PPFE on radiologic findings during the follow-up period.

CONCLUSION

Clinicians should consider PPFE, even in cases with unilateral, predominantly upper lung involvement in interstitial lung disease patients through multidisciplinary discussion.

Keywords: Interstitial lung disease, Pleuroparenchymal fibroelastosis, Progression, Unilateral, Multidisciplinary discussion, Case report

Core Tip: Pleuroparenchymal fibroelastosis (PPFE) has been classified among rare idiopathic interstitial pneumonia and is characterized by predominantly upper lobe involvement. However, there is increasing evidence for the coexistence of other interstitial lung diseases in the same lobe. Herein, we report a very rare case of PPFE with laterality, unilateral PPFE. The diagnosis of PPFE should be made after multidisciplinary discussion. In cases of unilateral upper lobe predominant fibrosis, physicians should consider the diagnosis of PPFE and identify the progression of PPFE after diagnosis.