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A Patient with Idiopathic Pleuroparenchymal Fibroelastosis Showing a Sustained Pulmonary Function due to Treatment with Pirfenidone
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- Sato Seidai
- Department of Respiratory Medicine & Rheumatology, Institute of Biomedical Sciences, Tokushima University Graduate School, Japan
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- Hanibuchi Masaki
- Department of Respiratory Medicine & Rheumatology, Institute of Biomedical Sciences, Tokushima University Graduate School, Japan
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- Takahashi Mikiko
- Department of Analytic Human Pathology, Nippon Medical School, Japan
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- Fukuda Yuh
- Department of Analytic Human Pathology, Nippon Medical School, Japan Division of Diagnostic Pathology, Itabashi Chuo Medical Center, Japan
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- Morizumi Shun
- Department of Respiratory Medicine & Rheumatology, Institute of Biomedical Sciences, Tokushima University Graduate School, Japan
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- Toyoda Yuko
- Department of Respiratory Medicine & Rheumatology, Institute of Biomedical Sciences, Tokushima University Graduate School, Japan
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- Goto Hisatsugu
- Department of Respiratory Medicine & Rheumatology, Institute of Biomedical Sciences, Tokushima University Graduate School, Japan
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- Nishioka Yasuhiko
- Department of Respiratory Medicine & Rheumatology, Institute of Biomedical Sciences, Tokushima University Graduate School, Japan
Bibliographic Information
- Other Title
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- A patient with idiopathic pleuroparenchymal fibroelastosis showing a sustained pulmonary function du to treatment with pirfenidone
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Description
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.<br>
Journal
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- Internal Medicine
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Internal Medicine 55 (5), 497-501, 2016
The Japanese Society of Internal Medicine
