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

被引：32

作者：

Sato, Seidai ^{[1
]}

Hanibuchi, Masaki ^{[1
]}

Takahashi, Mikiko ^{[2
]}

Fukuda, Yuh ^{[2
,3
]}

Morizumi, Shun ^{[1
]}

Toyoda, Yuko ^{[1
]}

Goto, Hisatsugu ^{[1
]}

Nishioka, Yasuhiko ^{[1
]}

机构：

[1] Univ Tokushima, Grad Sch, Inst Biomed Sci, Dept Resp Med & Rheumatol, Tokushima, Japan

[2] Nippon Med Sch, Dept Analyt Human Pathol, Tokyo, Japan

[3] Itabashi Chuo Med Ctr, Div Diagnost Pathol, Tokyo, Japan

来源：

INTERNAL MEDICINE | 2016年 / 55卷 / 05期

关键词：

idiopathic pleuroparenchymal fibroelastotis; idiopathic interstitial pneumonia; pirfenidone; pulmonary function; CLINICOPATHOLOGICAL ENTITY; FIBROSIS;

D O I：

10.2169/internalmedicine.55.5047

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

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.

引用

页码：497 / 501

页数：5

共 17 条

[1]

Amitani R., 1992, Kokyu, V11, P693

[2] Pirfenidone: Anti-fibrotic agent with a potential therapeutic role in the management of transplantation patients [J].

Dosanjh, A .

EUROPEAN JOURNAL OF PHARMACOLOGY, 2006, 536 (03) :219-222

[3] Quantitative analysis of lung elastic fibers in idiopathic pleuroparenchymal fibroelastosis (IPPFE): comparison of clinical, radiological, and pathological findings with those of idiopathic pulmonary fibrosis (IPF) [J].

Enomoto, Noriyuki ;

Kusagaya, Hideki ;

Oyama, Yoshiyuki ;

Kono, Masato ;

Kaida, Yusuke ;

Kuroishi, Shigeki ;

Hashimoto, Dai ;

Fujisawa, Tomoyuki ;

Yokomura, Koshi ;

Inui, Naoki ;

Nakamura, Yutaro ;

Suda, Takafumi .

BMC PULMONARY MEDICINE, 2014, 14

[4] Idiopathic pleuroparenchymal fibroelastosis - Description of a novel clinicopathologic entity [J].

Frankel, SK ;

Cool, CD ;

Lynch, DA ;

Brown, KK .

CHEST, 2004, 126 (06) :2007-2013

[5] The thoracic cage becomes flattened in the progression of pleuroparenchymal fibroelastosis [J].

Harada, Taishi ;

Yoshida, Yuji ;

Kitasato, Yasuhiko ;

Tsuruta, Nobuko ;

Wakamatsu, Kentaro ;

Hirota, Takako ;

Tanaka, Makoto ;

Tashiro, Naoki ;

Ishii, Hiroshi ;

Shiraishi, Motokimi ;

Fujita, Masaki ;

Nagata, Nobuhiko ;

Watanabe, Kentaro .

EUROPEAN RESPIRATORY REVIEW, 2014, 23 (132) :263-266

[6] A Phase 3 Trial of Pirfenidone in Patients with Idiopathic Pulmonary Fibrosis [J].

King, Talmadge E., Jr. ;

Bradford, Williamson Z. ;

Castro-Bernardini, Socorro ;

Fagan, Elizabeth A. ;

Glaspole, Ian ;

Glassberg, Marilyn K. ;

Gorina, Eduard ;

Hopkins, Peter M. ;

Kardatzke, David ;

Lancaster, Lisa ;

Lederer, David J. ;

Nathan, Steven D. ;

Pereira, Carlos A. ;

Sahn, Steven A. ;

Sussman, Robert ;

Swigris, Jeffrey J. ;

Noble, Paul W. .

NEW ENGLAND JOURNAL OF MEDICINE, 2014, 370 (22) :2083-2092

[7]

Kobayashi Y, 1999, Nihon Kokyuki Gakkai Zasshi, V37, P812

[8] Idiopathic pleuroparenchymal fibroelastosis: consideration of a clinicopathological entity in a series of Japanese patients [J].

Kusagaya, Hideki ;

Nakamura, Yutaro ;

Kono, Masato ;

Kaida, Yusuke ;

Kuroishi, Shigeki ;

Enomoto, Noriyuki ;

Fujisawa, Tomoyuki ;

Koshimizu, Naoki ;

Yokomura, Koshi ;

Inui, Naoki ;

Suda, Takafumi ;

Colby, Thomas V. ;

Chida, Kingo .

BMC PULMONARY MEDICINE, 2012, 12

[9]

Morimoto Akie, 2010, Nihon Kokyuki Gakkai Zasshi, V48, P944

[10]

Nei Takahito, 2009, Nihon Kokyuki Gakkai Zasshi, V47, P116

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