Clinical diagnosis of idiopathic pleuroparenchymal fibroelastosis

A retrospective multicenter study

Yasunori Enomoto, Yutaro Nakamura, Yasuomi Satake, Hiromitsu Sumikawa, Takeshi Johkoh, Thomas V. Colby, Hideki Yasui, Hironao Hozumi, Masato Karayama, Yuzo Suzuki, Kazuki Furuhashi, Tomoyuki Fujisawa, Noriyuki Enomoto, Naoki Inui, Toshihide Iwashita, Shigeki Kuroishi, Koshi Yokomura, Naoki Koshimizu, Mikio Toyoshima, Shiro Imokawa & 4 others Takashi Yamada, Toshihiro Shirai, Hiroshi Hayakawa, Takafumi Suda

Research output: Contribution to journalArticle

16 Citations (Scopus)

Abstract

Background Although the accurate diagnosis of pleuroparenchymal fibroelastosis (PPFE) requires pathologic evaluation, this diagnosis is often suggested when the radiologic findings are consistent with typical PPFE and when pulmonary apical cap, which radiologically and pathologically mimics PPFE, can be excluded by confirming disease progression. The aim of this study was to evaluate the validity of the clinical diagnosis of idiopathic PPFE. Methods We recruited 44 patients with idiopathic PPFE according to our modified diagnostic criteria: 1) a radiologic PPFE pattern (i.e., bilateral subpleural dense consolidation with or without pleural thickening in the upper lobes and less marked or absent involvement of the lower lobes), 2) radiologic confirmation of disease progression, and 3) exclusion of other lung diseases with identifiable etiologies. The patients’ baseline characteristics and clinical course were reviewed. Results The median age was 70 years, and 28 patients were males. The majority revealed emaciation, hypercapnia, and a high ratio of residual volume to total lung capacity. On chest computed tomography, 39 patients showed abnormal shadows in the lower lobes; more than half were classified as having usual interstitial pneumonia (UIP)/possible UIP pattern. Pneumothorax was the most frequent complication (33/44). The median overall survival time after diagnosis was 35.3 months. The presence of lower lobe UIP/possible UIP pattern did not show a significant prognostic impact. Conclusions Using our diagnostic criteria, we could recruit relatively many patients with similar characteristics to those of idiopathic PPFE patients in the literature. The possibility of clinical diagnosis of idiopathic PPFE should be further discussed.

Original languageEnglish (US)
Pages (from-to)1-5
Number of pages5
JournalRespiratory Medicine
Volume133
DOIs
StatePublished - Dec 1 2017

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Multicenter Studies
Idiopathic Pulmonary Fibrosis
Retrospective Studies
Disease Progression
Emaciation
Total Lung Capacity
Residual Volume
Hypercapnia
Pneumothorax
Lung Diseases
Thorax
Tomography
Lung
Survival

Keywords

  • Clinical diagnosis
  • Interstitial lung disease
  • Pleuroparenchymal fibroelastosis

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine

Cite this

Enomoto, Y., Nakamura, Y., Satake, Y., Sumikawa, H., Johkoh, T., Colby, T. V., ... Suda, T. (2017). Clinical diagnosis of idiopathic pleuroparenchymal fibroelastosis: A retrospective multicenter study. Respiratory Medicine, 133, 1-5. https://doi.org/10.1016/j.rmed.2017.11.003

Clinical diagnosis of idiopathic pleuroparenchymal fibroelastosis : A retrospective multicenter study. / Enomoto, Yasunori; Nakamura, Yutaro; Satake, Yasuomi; Sumikawa, Hiromitsu; Johkoh, Takeshi; Colby, Thomas V.; Yasui, Hideki; Hozumi, Hironao; Karayama, Masato; Suzuki, Yuzo; Furuhashi, Kazuki; Fujisawa, Tomoyuki; Enomoto, Noriyuki; Inui, Naoki; Iwashita, Toshihide; Kuroishi, Shigeki; Yokomura, Koshi; Koshimizu, Naoki; Toyoshima, Mikio; Imokawa, Shiro; Yamada, Takashi; Shirai, Toshihiro; Hayakawa, Hiroshi; Suda, Takafumi.

In: Respiratory Medicine, Vol. 133, 01.12.2017, p. 1-5.

Research output: Contribution to journalArticle

Enomoto, Y, Nakamura, Y, Satake, Y, Sumikawa, H, Johkoh, T, Colby, TV, Yasui, H, Hozumi, H, Karayama, M, Suzuki, Y, Furuhashi, K, Fujisawa, T, Enomoto, N, Inui, N, Iwashita, T, Kuroishi, S, Yokomura, K, Koshimizu, N, Toyoshima, M, Imokawa, S, Yamada, T, Shirai, T, Hayakawa, H & Suda, T 2017, 'Clinical diagnosis of idiopathic pleuroparenchymal fibroelastosis: A retrospective multicenter study', Respiratory Medicine, vol. 133, pp. 1-5. https://doi.org/10.1016/j.rmed.2017.11.003
Enomoto, Yasunori ; Nakamura, Yutaro ; Satake, Yasuomi ; Sumikawa, Hiromitsu ; Johkoh, Takeshi ; Colby, Thomas V. ; Yasui, Hideki ; Hozumi, Hironao ; Karayama, Masato ; Suzuki, Yuzo ; Furuhashi, Kazuki ; Fujisawa, Tomoyuki ; Enomoto, Noriyuki ; Inui, Naoki ; Iwashita, Toshihide ; Kuroishi, Shigeki ; Yokomura, Koshi ; Koshimizu, Naoki ; Toyoshima, Mikio ; Imokawa, Shiro ; Yamada, Takashi ; Shirai, Toshihiro ; Hayakawa, Hiroshi ; Suda, Takafumi. / Clinical diagnosis of idiopathic pleuroparenchymal fibroelastosis : A retrospective multicenter study. In: Respiratory Medicine. 2017 ; Vol. 133. pp. 1-5.
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T1 - Clinical diagnosis of idiopathic pleuroparenchymal fibroelastosis

T2 - A retrospective multicenter study

AU - Enomoto, Yasunori

AU - Nakamura, Yutaro

AU - Satake, Yasuomi

AU - Sumikawa, Hiromitsu

AU - Johkoh, Takeshi

AU - Colby, Thomas V.

AU - Yasui, Hideki

AU - Hozumi, Hironao

AU - Karayama, Masato

AU - Suzuki, Yuzo

AU - Furuhashi, Kazuki

AU - Fujisawa, Tomoyuki

AU - Enomoto, Noriyuki

AU - Inui, Naoki

AU - Iwashita, Toshihide

AU - Kuroishi, Shigeki

AU - Yokomura, Koshi

AU - Koshimizu, Naoki

AU - Toyoshima, Mikio

AU - Imokawa, Shiro

AU - Yamada, Takashi

AU - Shirai, Toshihiro

AU - Hayakawa, Hiroshi

AU - Suda, Takafumi

PY - 2017/12/1

Y1 - 2017/12/1

N2 - Background Although the accurate diagnosis of pleuroparenchymal fibroelastosis (PPFE) requires pathologic evaluation, this diagnosis is often suggested when the radiologic findings are consistent with typical PPFE and when pulmonary apical cap, which radiologically and pathologically mimics PPFE, can be excluded by confirming disease progression. The aim of this study was to evaluate the validity of the clinical diagnosis of idiopathic PPFE. Methods We recruited 44 patients with idiopathic PPFE according to our modified diagnostic criteria: 1) a radiologic PPFE pattern (i.e., bilateral subpleural dense consolidation with or without pleural thickening in the upper lobes and less marked or absent involvement of the lower lobes), 2) radiologic confirmation of disease progression, and 3) exclusion of other lung diseases with identifiable etiologies. The patients’ baseline characteristics and clinical course were reviewed. Results The median age was 70 years, and 28 patients were males. The majority revealed emaciation, hypercapnia, and a high ratio of residual volume to total lung capacity. On chest computed tomography, 39 patients showed abnormal shadows in the lower lobes; more than half were classified as having usual interstitial pneumonia (UIP)/possible UIP pattern. Pneumothorax was the most frequent complication (33/44). The median overall survival time after diagnosis was 35.3 months. The presence of lower lobe UIP/possible UIP pattern did not show a significant prognostic impact. Conclusions Using our diagnostic criteria, we could recruit relatively many patients with similar characteristics to those of idiopathic PPFE patients in the literature. The possibility of clinical diagnosis of idiopathic PPFE should be further discussed.

AB - Background Although the accurate diagnosis of pleuroparenchymal fibroelastosis (PPFE) requires pathologic evaluation, this diagnosis is often suggested when the radiologic findings are consistent with typical PPFE and when pulmonary apical cap, which radiologically and pathologically mimics PPFE, can be excluded by confirming disease progression. The aim of this study was to evaluate the validity of the clinical diagnosis of idiopathic PPFE. Methods We recruited 44 patients with idiopathic PPFE according to our modified diagnostic criteria: 1) a radiologic PPFE pattern (i.e., bilateral subpleural dense consolidation with or without pleural thickening in the upper lobes and less marked or absent involvement of the lower lobes), 2) radiologic confirmation of disease progression, and 3) exclusion of other lung diseases with identifiable etiologies. The patients’ baseline characteristics and clinical course were reviewed. Results The median age was 70 years, and 28 patients were males. The majority revealed emaciation, hypercapnia, and a high ratio of residual volume to total lung capacity. On chest computed tomography, 39 patients showed abnormal shadows in the lower lobes; more than half were classified as having usual interstitial pneumonia (UIP)/possible UIP pattern. Pneumothorax was the most frequent complication (33/44). The median overall survival time after diagnosis was 35.3 months. The presence of lower lobe UIP/possible UIP pattern did not show a significant prognostic impact. Conclusions Using our diagnostic criteria, we could recruit relatively many patients with similar characteristics to those of idiopathic PPFE patients in the literature. The possibility of clinical diagnosis of idiopathic PPFE should be further discussed.

KW - Clinical diagnosis

KW - Interstitial lung disease

KW - Pleuroparenchymal fibroelastosis

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