A Patient with Idiopathic Pleuroparenchymal Fibroelastosis Showing a Sustained Pulmonary Function due to Treatment with Pirfenidone

Seidai Sato; Masaki Hanibuchi; Mikiko Takahashi; Yuh Fukuda; Shun Morizumi; Yuko Toyoda; Hisatsugu Goto; Yasuhiko Nishioka

doi:10.2169/internalmedicine.55.5047

CASE REPORTS

A Patient with Idiopathic Pleuroparenchymal Fibroelastosis Showing a Sustained Pulmonary Function due to Treatment with Pirfenidone

Seidai Sato, Masaki Hanibuchi, Mikiko Takahashi, Yuh Fukuda, Shun Morizumi, Yuko Toyoda, Hisatsugu Goto, Yasuhiko Nishioka

Author information

Seidai Sato
Department of Respiratory Medicine & Rheumatology, Institute of Biomedical Sciences, Tokushima University Graduate School, Japan
Masaki Hanibuchi
Department of Respiratory Medicine & Rheumatology, Institute of Biomedical Sciences, Tokushima University Graduate School, Japan
Mikiko Takahashi
Department of Analytic Human Pathology, Nippon Medical School, Japan
Yuh Fukuda
Department of Analytic Human Pathology, Nippon Medical School, Japan
Division of Diagnostic Pathology, Itabashi Chuo Medical Center, Japan
Shun Morizumi
Department of Respiratory Medicine & Rheumatology, Institute of Biomedical Sciences, Tokushima University Graduate School, Japan
Yuko Toyoda
Department of Respiratory Medicine & Rheumatology, Institute of Biomedical Sciences, Tokushima University Graduate School, Japan
Hisatsugu Goto
Department of Respiratory Medicine & Rheumatology, Institute of Biomedical Sciences, Tokushima University Graduate School, Japan
Yasuhiko Nishioka
Department of Respiratory Medicine & Rheumatology, Institute of Biomedical Sciences, Tokushima University Graduate School, Japan

Keywords: idiopathic pleuroparenchymal fibroelastotis, idiopathic interstitial pneumonia, pirfenidone, pulmonary function

JOURNAL OPEN ACCESS

2016 Volume 55 Issue 5 Pages 497-501

DOI https://doi.org/10.2169/internalmedicine.55.5047

Details

Abstract

The patient was a 68-year-old man presenting with body weight loss and exertional dyspnea. High-resolution computed tomography of the chest showed dense subpleural consolidation with traction bronchiectasis and volume loss predominantly in bilateral apical lesions and upper lobes. A histopathological analysis of a specimen of the right upper lobe showed histological patterns which were consistent with idiopathic pleuroparenchymal fibroelastotis (IPPFE). Treatment with pirfenidone was introduced with the expectation of its potential benefit. The effect of pirfenidone was satisfactory, and a decline in forced vital capacity was inhibited during treatment. This is the first case report suggesting the efficacy of pirfenidone for patients with IPPFE.

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