Lung Transplantation for Pleuroparenchymal Fibroelastosis After Chemotherapy

Chen, Fengshi; Matsubara, Kousaku; Miyagawa‐Hayashino, Aya; Tada, Kimihide; Handa, Tomohiro; Yamada, Tetsu; Sato, Masaaki; Aoyama, Akihiro; Date, Hiroshi

doi:10.1016/j.athoracsur.2014.07.045

Cited by 40 publications

(29 citation statements)

References 7 publications

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“…Infection control should be a priority. In advanced disease, it is appropriate to consider lung transplantation, which as been successfully performed in a handful of reported cases …”

Section: Ippfementioning

confidence: 99%

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Rare idiopathic interstitial pneumonias: LIP and PPFE and rare histologic patterns of interstitial pneumonias: AFOP and BPIP

et al. 2015

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In the 2013 reclassification of the idiopathic interstitial pneumonias (IIPs), two rare IIPs (idiopathic lymphoid interstitial pneumonia (LIP), idiopathic pleuroparenchymal fibroelastosis (IPPFE)) and two rare histologic patterns (acute fibrinous and organizing pneumonia (AFOP), bronchiolocentric pattern of interstitial pneumonia (BPIP)) are described. All these entities are rare with small series published to date, mostly containing primary and secondary forms of disease. LIP is histologically characterized by diffuse polyclonal lymphoid cell infiltrate surrounding the airways and expanding the interstitium. Thin-walled cysts and diffuse ground glass are considered the typical radiologic features. The clinical course is highly variable with corticosteroid responsiveness evident in approximately half of cases. IPPFE is defined histologically by coexisting upper lobe pleural and intraalveolar fibrosis with elastosis. Dense subpleural irregular fibrosis and consolidation are the cardinal radiologic features. A history of recurrent lower respiratory tract infection is frequent. Responses to immunomodulation have not been reported and the rate of progression appears to be highly variable. AFOP is a rare histologic pattern lying within the spectrum of acute/subacute lung injury, characterized by organizing pneumonia and intra-alveolar fibrin deposition without hyaline membranes. BPIP is characterized histologically by fibrosis and/or inflammation confined to the alveolar interstitium around bronchovascular bundles, overlapping with peribronchial metaplasia and fibrosis in some series. Currently, AFOP and BPIP are both best viewed as histological entities rather than true clinical disorders, in the absence of characteristic associated imaging patterns and clinical features.

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“…Infection control should be a priority. In advanced disease, it is appropriate to consider lung transplantation, which as been successfully performed in a handful of reported cases …”

Section: Ippfementioning

confidence: 99%

“…In advanced disease, it is appropriate to consider lung transplantation, which as been successfully performed in a handful of reported cases. 37,50,51 AFOP AFOP is characterized typically by intra-alveolar organizing pneumonia with coexistent fibrin deposition and a lack of hyaline membranes. The process is usually patchy 1 (Fig.…”

Section: Ippfementioning

confidence: 99%

Rare idiopathic interstitial pneumonias: LIP and PPFE and rare histologic patterns of interstitial pneumonias: AFOP and BPIP

et al. 2015

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“…Of ILDs with PPFE lesions, idiopathic PPFE and LONIPC are often progressive and fatal [2, 3, 10, 12, 13], but the rate of disease progression is variable [14]. Idiopathic pulmonary fibrosis (IPF) with pathological PPFE also tends to have worse survival than IPF without pathological PPFE [5].…”

Section: Introductionmentioning

confidence: 99%

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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“…Due to the heterogeneous etiology and the presenting symptoms which masquerade as common complaints, time from symptom onset to diagnosis has been reported anywhere between 1 and 16 years in adults and the only curative approach is lung transplantation as medical approaches have not been shown to be effective 3,4 . Our patient was not diagnosed until 2 years after the onset of her cough, and an earlier recognition of PPFE may have made treatment by lung transplant feasible 9 . Histopathology continues to be the gold standard for diagnosing PPFE, but lung biopsy is fraught with complications.…”

Section: Discussionmentioning

confidence: 82%

Pleuroparenchymal fibroelastosis in an 8‐year old treated for neuroblastoma

Gerstle

Tanager

Hegde

et al. 2020

Pediatric Pulmonology

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Pleuroparenchymal fibroelastosis (PPFE), which is primarily diagnosed in adults, is a progressive lung pathology associated with significant morbidity and mortality. PPFE is characterized by pleural and subpleural parenchymal disease causing dyspnea, cough, and recurrent pneumothoraces. PPFE can be precipitated by autoimmune disorders, recurrent respiratory infections, chemotherapy, and transplant. We describe the youngest recorded patient to develop PPFE, whose symptoms began several years after treatment for neuroblastoma. Her symptoms were initially mistaken for worsening asthma, and multiple comorbidities developed during the prolonged time to recognition of PPFE and she progressed to fatal lung disease before potentially curative lung transplantation could occur.

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Lung Transplantation for Pleuroparenchymal Fibroelastosis After Chemotherapy

Cited by 40 publications

References 7 publications

Rare idiopathic interstitial pneumonias: LIP and PPFE and rare histologic patterns of interstitial pneumonias: AFOP and BPIP

Rare idiopathic interstitial pneumonias: LIP and PPFE and rare histologic patterns of interstitial pneumonias: AFOP and BPIP

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

Pleuroparenchymal fibroelastosis in an 8‐year old treated for neuroblastoma

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