Clinical diagnosis of idiopathic pleuroparenchymal fibroelastosis: A retrospective multicenter study

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 exclud...

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Bibliographic Details
Published in:Respiratory medicine 2017-12, Vol.133, p.1-5
Main Authors: 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
Format: Article
Language:English
Subjects:
Aged
Clinical diagnosis
Computed tomography
Consolidation
Criteria
Diagnosis
Diagnostic systems
Disease Progression
Elastic Tissue - pathology
Etiology
Family medical history
Female
Humans
Hypercapnia
Hypercapnia - diagnosis
Interstitial lung disease
Japan - epidemiology
Lobes
Lung - diagnostic imaging
Lung - pathology
Lung - physiopathology
Lung cancer
Lung diseases
Lung Diseases, Interstitial - diagnosis
Lung Diseases, Interstitial - diagnostic imaging
Lung Diseases, Interstitial - pathology
Lung Diseases, Interstitial - physiopathology
Male
Males
Medical diagnosis
Medical prognosis
Patients
Pleura - pathology
Pleural Diseases - complications
Pleural Diseases - diagnostic imaging
Pleural Diseases - pathology
Pleuroparenchymal fibroelastosis
Pneumonia
Pneumothorax
Pneumothorax - complications
Prognosis
Pulmonary fibrosis
Pulmonary Fibrosis - complications
Pulmonary Fibrosis - diagnostic imaging
Pulmonary Fibrosis - pathology
Radiography
Retrospective Studies
Sarcoidosis
Thickening
Tomography, X-Ray Computed
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Summary: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. 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. 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. 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. •We propose criteria to clinically diagnose pleuroparenchymal fibroelastosis (PPFE).•According to them, 44 patients were recruited regardless of the pathologic evidence.•Their clinical findings were similar to those of idiopathic PPFE in the literature.•Idiopathic PPFE can be diagnosed even without pathologic evaluation.
ISSN:0954-6111
1532-3064
DOI:10.1016/j.rmed.2017.11.003