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Pleuroparenchymal fibroelastosis in Korean patients: clinico-radiologic-pathologic features and 2-year follow-up

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Abstract
Background/Aims: Pleuroparenchymal fibroelastosis (PPFE) is a type of rare idiopathic interstitial pneumonia that is characterized by predominantly upper lobe involvement with pleural fibrosis and subjacent parenchymal fibrosis. This study aimed to determine the clinico-radiologic-pathologic features and prognosis of Korean patients with PPFE.

Methods: A total of 26 patients who were confirmed to have PPFE by lung biopsy, were included, and their clinico-radiologic-pathologic findings were retrospectively analyzed.

Results: The mean follow-up period was 23.8 months, and the mean age of the patients was 62.5 years; 61.5% were men and 50% were smokers. Cough and dyspnea were the most frequent presenting symptoms, and restrictive pattern was the most common observation in lung function. In 84.6% of the subjects, lower lobe involvement was found on chest computed tomography, and the usual interstitial pneumonia (UIP) pattern was the most common (59.1%). Among patients whose lower lobe was biopsied (n = 13), the UIP pattern was the most common (46.2%). Patients with lower lobe involvement were older and walked a shorter distance during the 6-minute walk test, compared to those without. Spontaneous pneumothorax was a common complication (26.9%), and 15.4% of the patients died mostly due to pneumonia (ioo%). The 1- and 3-year survival rates were 90.2% and 84.5%, respectively.

Conclusions: Clinical features of Korean patients with PPFE were similar to those reported previously; however, lower lobe involvement was more frequent. During follow-up, one-fourth of the patients experienced pneumothorax and one-fifth died from pneumonia.
Author(s)
송준선송진우채은진Jae Ha LeeMiae Kim
Issued Date
2021
Type
Article
Keyword
idiopathic interstitial pneumoniaspleural diseasespulmonary fibrosissurvival analysis내과학
DOI
10.3904/kjim.2019.303
URI
https://oak.ulsan.ac.kr/handle/2021.oak/7044
https://ulsan-primo.hosted.exlibrisgroup.com/primo-explore/fulldisplay?docid=TN_cdi_nrf_kci_oai_kci_go_kr_ARTI_9745112&context=PC&vid=ULSAN&lang=ko_KR&search_scope=default_scope&adaptor=primo_central_multiple_fe&tab=default_tab&query=any,contains,Pleuroparenchymal%20fibroelastosis%20in%20Korean%20patients:%20clinico-radiologic-pathologic%20features%20and%202-year%20follow-up&offset=0&pcAvailability=true
Publisher
KOREAN JOURNAL OF INTERNAL MEDICINE
Location
대한민국
Language
영어
ISSN
1226-3303
Citation Volume
36
Citation Number
1
Citation Start Page
132
Citation End Page
141
Appears in Collections:
Medicine > Medicine
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