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

doi:10.1016/j.rmed.2017.11.003

Cited by 101 publications

(154 citation statements)

References 18 publications

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“…In our study, we classified patients with only definite UIP pattern into the PPFE and UIP group. The difference in survival between the prior publication and our research is the difference in UIP classification between definite UIP only in our research and both definite and possible UIP pattern in Enomoto et al [18] research.…”

Section: Discussioncontrasting

confidence: 64%

“…Our study suggested that a definite UIP pattern was a risk factor for short survival in PPFE. In contrast, it was reported that there was no significant difference in survival time between the patients with and without definite/possible UIP on HRCT by Enomoto et al [18]. In this study, they did not evaluate differences in prognosis between patients with definite UIP and with possible UIP.…”

Section: Discussionmentioning

confidence: 62%

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Usual Interstitial Pneumonia Pattern in the Lower Lung Lobes as a Prognostic Factor in Idiopathic Pleuroparenchymal Fibroelastosis

et al. 2018

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Background: Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a rare interstitial pneumonia that is characterized by stiffness in both the upper lobes and pleura, which is evident on high resolution computed tomography (HRCT) of the chest. However, prognostic factors for IPPFE have not been identified yet. Objective: We aimed to investigate the clinical prognostic factors affecting survival in patients with IPPFE. Methods: Between April 2009 and September 2017, we enrolled 36 patients who were clinically diagnosed with IPPFE, using HRCT. These patients were classified as either short survival (dead within 12 months from the diagnosis of IPPFE) or long survival (survived for greater than 12 months) groups. We retrospectively analyzed the clinical characteristics, serum markers, pulmonary function test results, and HRCT findings. Results: Twelve patients were classified into the short survival and 24 were categorized into long survival categories. At the time of diagnosis, the incidence of coexistence of a usual interstitial pneumonia (UIP) pattern in the lower lobes on HRCT in the short survival was significantly higher than that in the long survival. Multivariate analysis revealed that a UIP pattern in the lower lobes on HRCT was the only independent variable for poor prognosis. The median survival time from diagnosis in patients with IPPFE was 24 months. Of these patients with IPPFE, the survival time with a UIP pattern was significantly shorter than in those without a UIP pattern. Conclusion: Our findings suggest that a UIP pattern in the lower lobes at the time of diagnosis was an independent prognostic factor for IPPFE.

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Section: Discussioncontrasting

confidence: 64%

Section: Discussionmentioning

confidence: 62%

Usual Interstitial Pneumonia Pattern in the Lower Lung Lobes as a Prognostic Factor in Idiopathic Pleuroparenchymal Fibroelastosis

et al. 2018

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“…History of pneumothorax, low BMI, and low %FVC were previously reported for patients with idiopathic PPFE, LONIPC, and IPF with pathological PPFE [1, 5, 6, 15, 19]. Restrictive impairment present in patients with radiological PPFE may be partially caused by a flattened thoracic cage, in addition to lung parenchymal fibrosis.…”

Section: Discussionmentioning

confidence: 91%

“…Differentiation of radiological PPFE from apical cap may be difficult in some cases [12]. A recent study included radiological progression of disease in the diagnostic criteria for idiopathic PPFE to exclude apical cap [19]. All radiological PPFE cases in our cohort showed traction bronchiectasis or volume loss of the upper lobes, suggesting disease progression different from apical cap.…”

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 as a late complication of childhood cancer therapy: A case series

Marathe,

Santibanez,

Meyers

et al. 2024

Pediatric Blood & Cancer

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Pleuroparenchymal fibroelastosis (PPFE) is a rare interstitial pneumonia with distinct clinicopathologic features. It has been associated with exposure to hematopoietic stem cell transplantation (HSCT) and classical alkylating agents. Here, we highlight PPFE as a late complication of childhood cancer therapy by describing the cases of four survivors of childhood cancer with a diagnosis of treatment‐related PPFE. All patients received high‐dose alkylating agents. PPFE should be considered in the differential diagnosis of restrictive lung disease in patients with history of exposure to alkylating agents or HSCT. Development of PPFE‐specific, noninvasive diagnostic tools and disease‐modifying therapies will clinically benefit these patients.

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Clinical diagnosis of idiopathic pleuroparenchymal fibroelastosis: A retrospective multicenter study

Abstract: 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.

Cited by 101 publications

References 18 publications

Usual Interstitial Pneumonia Pattern in the Lower Lung Lobes as a Prognostic Factor in Idiopathic Pleuroparenchymal Fibroelastosis

Usual Interstitial Pneumonia Pattern in the Lower Lung Lobes as a Prognostic Factor in 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 as a late complication of childhood cancer therapy: A case series

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