Heterogeneous clinical features in patients with pulmonary fibrosis showing histology of pleuroparenchymal fibroelastosis

Yoshida, Yūji; Nagata, Nobuhiko; Tsuruta, Nobuko; Kitasato, Yasuhiko; Wakamatsu, Kentaro; Yoshimi, Michihiro; Ishii, Hiroshi; Hirota, Takako; Hamada, Naoki; Fujita, Masaki; Nabeshima, Kazuki; Kiyomi, Fumiaki; Watanabe, Kentaro

doi:10.1016/j.resinv.2015.11.002

Cited by 54 publications

(79 citation statements)

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“…Extension of a PPFE pattern to the lower lobes has been demonstrated in a subgroup of patients [21]. These data have been recently confirmed in a relatively large series of Japanese patients ( n = 21), reporting an equal involvement of upper and lower lobes in approximately one third of cases [22]. …”

Section: The Clinical Spectrummentioning

confidence: 76%

“…Due to the long subclinical phase, IPPFE is commonly diagnosed in the advanced stages, making a reliable assessment of disease behavior since its first onset difficult [2]. Yoshida et al have recently identified two distinct patterns of lung function decline in their cohort of patients, a rapid decline in FVC over a short period, and a slow decline over a longer period, although the latter was observed only in one quarter of subjects [22]. …”

Section: The Clinical Spectrummentioning

confidence: 99%

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Idiopathic Pleuroparenchymal Fibroelastosis

2017

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Purpose of the reviewIdiopathic pleuroparenchymal fibroelastosis (IPPFE) is a rare fibrosing lung disease, affecting the visceral pleura and the subpleural parenchyma with an upper lobe predilection, included as a distinct clinicopathologic entity in the latest international multidisciplinary classification of the idiopathic interstitial pneumonias (IIP). We aim to summarize the current evidence on IPPFE, in terms of clinical features and potential treatments.Recent findingsOverall, there is increasing awareness of PPFE in association with a separate ILD pattern. Although an agreed consensus on diagnosis has yet to be defined, a list of radiological and histopathological criteria has been proposed. Due to the unfavorable risk-benefit profile of surgical lung biopsy in a significant proportion of patients, a potential role for transbronchial lung cryobiopsy has been suggested. At present, lung transplantation remains the only curative option.SummaryThe increasing awareness of this condition among specialists has led to more frequent identification of IPPFE. Large international studies are needed to better characterize pathogenesis and pheno/endotypes of disease, a key step towards the development of effective treatments.

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Section: The Clinical Spectrummentioning

confidence: 76%

Section: The Clinical Spectrummentioning

confidence: 99%

Idiopathic Pleuroparenchymal Fibroelastosis

2017

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“…In idiopathic PPFE/LONIPC, PPFE lesions are predominant, and these lesions worsen at variable rates of progression [14]. In other fibrotic ILDs with radiological PPFE, other lesions than PPFE, such as UIP and NSIP, are predominant, and they may influence survival independently from PPFE.…”

Section: Discussionmentioning

confidence: 99%

“…Some physicians may have given up referring the most severe cases to LT centers, whether or not the cases have radiological PPFE. Two phenotypes of disease progression (rapid and slow) were reported in idiopathic PPFE [14]. It is possible that our cohort mostly included patients with slowly progressive idiopathic PPFE, not another rapidly progressive phenotype, which can be associated with better survival of idiopathic PPFE and LONIPC with radiological PPFE.…”

Section: Discussionmentioning

confidence: 99%

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Clinical significance of radiological pleuroparenchymal fibroelastosis pattern in interstitial lung disease patients registered for lung transplantation: a retrospective cohort study

et al. 2018

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BackgroundRadiological pleuroparenchymal fibroelastosis (PPFE) lesion is characterized by pleural thickening with associated signs of subpleural fibrosis on high-resolution computed tomography (HRCT). This study evaluated the clinical significance of radiological PPFE as an isolated finding or associated with other interstitial lung diseases (ILDs) in patients having fibrotic ILDs and registered for cadaveric lung transplantation (LT).MethodsThis retrospective study included 118 fibrotic ILD patients registered for LT. Radiological PPFE on HRCT was assessed. The impact of radiological PPFE on clinical features and transplantation-censored survival were evaluated.ResultsRadiological PPFE was observed in 30/118 cases (25%): definite PPFE (PPFE concentrated in the upper lobes, with involvement of lower lobes being less marked) in 12 (10%) and consistent PPFE (PPFE not concentrated in the upper lobes, or PPFE with features of coexistent disease present elsewhere) in 18 (15%). Of these, 12 had late-onset non-infectious pulmonary complications after hematopoietic stem-cell transplantation and/or chemotherapy (LONIPCs), 9 idiopathic PPFE, and 9 other fibrotic ILDs (idiopathic pulmonary fibrosis, IPF; other idiopathic interstitial pneumonias, other IIPs; connective tissue disease-associated ILD, CTD-ILD, and hypersensitivity pneumonia, HP). Radiological PPFE was associated with previous history of pneumothorax, lower body mass index, lower percentage of predicted forced vital capacity (%FVC), higher percentage of predicted diffusion capacity of carbon monoxide, less desaturation on six-minute walk test, and hypercapnia. The median survival time of all study cases was 449 days. Thirty-seven (28%) received LTs: cadaveric in 31 and living-donor lobar in six. Of 93 patients who did not receive LT, 66 (71%) died. Radiological PPFE was marginally associated with better survival after adjustment for age, sex, %FVC, and six-minute walk distance < 250 m (hazard ratio 0.51 [0.25–1.05], p = 0.07). After adjustment for covariates, idiopathic PPFE and LONIPC with radiological PPFE was associated with better survival than fibrotic ILDs without radiological PPFE (hazard ratio 0.38 [0.16–0.90], p = 0.03), and marginally better survival than other fibrotic ILDs with radiological PPFE (hazard ratio, 0.20 [0.04–1.11], p = 0.07).Conclusionsidiopathic PPFE and LONIPC with radiological PPFE has better survival on the wait list for LT than fibrotic ILDs without radiological PPFE, after adjustment for age, sex, %FVC, and six-minute walk distance.Electronic supplementary materialThe online version of this article (10.1186/s12931-018-0860-6) contains supplementary material, which is available to authorized users.

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Pleuroparenchymal Fibroelastosis

Suda

2023

Orphan Lung Diseases

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Heterogeneous clinical features in patients with pulmonary fibrosis showing histology of pleuroparenchymal fibroelastosis

Cited by 54 publications

References 22 publications

Idiopathic Pleuroparenchymal Fibroelastosis

Idiopathic Pleuroparenchymal Fibroelastosis

Clinical significance of radiological pleuroparenchymal fibroelastosis pattern in interstitial lung disease patients registered for lung transplantation: a retrospective cohort study

Pleuroparenchymal Fibroelastosis

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