Pleuroparenchymal Fibroelastosis of the Lung: A Review

Cheng, Shaun Kian Hong; Chuah, Khoon Leong

doi:10.5858/arpa.2015-0166-rs

Cited by 53 publications

(59 citation statements)

References 14 publications

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“…IPPFE usually presents in adults, but the age at onset is highly heterogeneous, ranging from 13 to 87 years, with a median value of approximately 53 years [13]. In particular, a bimodal distribution of age at presentation has been observed, with an earlier peak in the third decade, and a later one in the sixth decade.…”

Section: The Clinical Spectrummentioning

confidence: 99%

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: 99%

Idiopathic Pleuroparenchymal Fibroelastosis

2017

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“…[2] PPFE is characterized radiologically by apical pleural thickening and histologically by elastic fibrosis of the visceral pleura. [2,3] In 2013, iPPFE was defined to be a subtype of rare idiopathic interstitial pneumonia. [1] Studies about PPFE have occasionally been published, and more than 100 PPFE cases have been reported.…”

Section: Discussionmentioning

confidence: 99%

“…[1] Studies about PPFE have occasionally been published, and more than 100 PPFE cases have been reported. [2,4–9] Most of these cases involved PPFE secondary to organ or stem cell transplantation, chemotherapy, autoimmune disease, or recurrent infection. [2,4,10] iPPFE cases have been less thoroughly studied.…”

Section: Discussionmentioning

confidence: 99%

“…[1] There is no effective treatment for primary or secondary PPFE. [2] We report successful bilateral lung transplantation in a 36-year-old male with iPPFE after the failure of treatment with prednisone plus cyclophosphamide and sequential pirfenidone administration. Written informed consent for the case report was obtained from this patient, and the consent procedure was approved by the Ethics Committee of the Peking Union Medical College Hospital.…”

Section: Introductionmentioning

confidence: 99%

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A CARE-compliant case report

Huang¹,

Feng

Li³

et al. 2017

Medicine

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Rational:Pleuroparenchymal fibroelastosis (PPFE) is a rare interstitial lung disease that is characterized radiologically by apical pleural thickening and histologically by elastic fibrosis of the visceral pleura. Although PPFE cases have been reported occasionally since this disease was initially described, most such cases have involved secondary PPFE. Idiopathic PPFE (iPPFE) cases have been less thoroughly studied. There are no effective medications for patients with iPPFE.Patient Concerns:A 34-year-old man with no asbestos or cigarette exposure was admitted to our ward due to worsening cough and exertional dyspnea for 10 years. He had a “flattened thoracic cage” and bibasilar inspiratory crackles without finger clubbing. A series of chest computed tomography scans during the preceding 10 years revealed the presence of gradual, exaggerated, upper lung–predominant, diffuse pleural thickening and dense subpleural opacification with traction bronchiectasis.Diagnosis:He was performed with video-assisted thoracic surgical (VATS) lung biopsy. The pulmonary histopathologic examination showed thickened visceral pleura and prominent subpleural fibroelastosis, confirming the diagnosis of iPPFE.Intervention:After the failure of treatment with prednisone plus cyclophosphamide and sequential pirfenidone administration, he was arranged with bilateral lung transplantation two years later.Outcomes:The patient did not require supplemental oxygenation anymore after he recovered from lung transplantation.Lessons:Bilateral lung transplantation might be tried for the end-stage iPPFE cases.

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“…Pleuroparenchymal fibroelastosis (PPFE) is a rare form of upper-lobe-dominant progressive pulmonary fibrosis characterized histologically by visceral pleural thickening with collagenous fibrosis, subpleural elastosis, and intra-alveolar collagenous fibrosis [1,2]. It was first described 25 years ago by Amitani and Kuse [2].…”

mentioning

confidence: 99%

Airway-Centered Pleuroparenchymal Fibroelastosis Associated with Non-Necrotizing Granulomas: A Rare New Entity

Bargagli¹,

Rottoli²,

Torricelli³

et al. 2018

Pathobiology

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Pleuroparenchymal fibroelastosis is a rare form of upper-lobe-dominant progressive pulmonary fibrosis characterized histologically by visceral pleural thickening with collagenous fibrosis, subpleural elastosis, and intra-alveolar collagenous fibrosis. It was first described 25 years ago by Amitani et al. This report firstly describes a new variant or rare phenotype of PPFE with airway involvement, minimal pleuroparenchymal connections, and non-necrotizing granulomas.

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Pleuroparenchymal Fibroelastosis of the Lung: A Review

Cited by 53 publications

References 14 publications

Idiopathic Pleuroparenchymal Fibroelastosis

Idiopathic Pleuroparenchymal Fibroelastosis

A CARE-compliant case report

Airway-Centered Pleuroparenchymal Fibroelastosis Associated with Non-Necrotizing Granulomas: A Rare New Entity

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