Special Issue "Pleuroparenchymal Fibroelastosis: Obstacles and Challenges"

A special issue of Journal of Clinical Medicine (ISSN 2077-0383). This special issue belongs to the section "Pulmonology".

Deadline for manuscript submissions: closed (30 June 2021) | Viewed by 7347

Special Issue Editor

Prof. Dr. Hiroshi Ishii
E-Mail Website
Guest Editor
Department of Respiratory Medicine, Fukuoka University Chikushi Hospital, Fukuoka, Japan
Interests: interstitial pneumonia; pulmonary fibrosis; connective tissue disease-related interstitial lung disease

Special Issue Information

Dear Colleagues,

The field of pleuroparenchymal fibroelastosisis (PPFE) research is growing rapidly. PPFE is a rare interstitial pneumonia consisting of alveolar septal elastosis and intra-alveolar collagenosis, predominantly located in the upper lobes. Recent discoveries suggest that the clinical and physiological characteristics of upper lobe fibroelastosis differ from those of idiopathic pulmonary fibrosis (IPF), including a flattened thoracic cage and a marked decrease in forced vital capacity, but with preserved residual volume. The prognosis may be related to the development of fibrosing interstitial pneumonia in the lower lobes. However, there is marked variation in the pathogenesis and clinical features of PPFE. Although the diagnostic criteria for PPFE are based on histological findings by surgical lung biopsy (SLB), we have limited chances to perform an SLB for the diagnosis of PPFE. A proposal concerning the diagnostic criteria for idiopathic PPFE with and without an SLB was recently published. However, there are no established blood biomarkers to aid in the diagnosis or prediction of a progressive phenotype in PPFE patients. Mechanisms of the occurrence and evolution of fibroelastosis in PPFE are still unknown. In addition, treatment strategies for PPFE, targeting the inhibition of elastosis, have not been established. The present Special Issue aims to deepen our understanding of the characteristics of PPFE to increase our knowledge of a, so far, poorly explored research area, and to seek innovative treatment of PPFE.

Prof. Dr. Hiroshi Ishii
Guest Editor

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Keywords

  • peuroparenchymal fibroelastosis
  • clinical and physiological findings
  • prognostic predictor
  • histological characteristics
  • predisposing factors or comorbidities
  • pathogenic mechanism
  • biomarker/molecular target

Published Papers (6 papers)

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Research

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Article
Prognostic and Clinical Value of Cluster Analysis in Idiopathic Pleuroparenchymal Fibroelastosis Phenotypes
J. Clin. Med. 2021, 10(7), 1498; https://doi.org/10.3390/jcm10071498 - 04 Apr 2021
Cited by 3 | Viewed by 1043
Abstract
Idiopathic pleuroparenchymal fibroelastosis (PPFE) is a distinctive interstitial pneumonia with upper lobe predominance that shows unique morphological features among idiopathic interstitial pneumonias (IIPs). Affected patients have a variety of clinical presentations with heterogeneous clinical courses. Cluster analysis is a valuable tool for identifying [...] Read more.
Idiopathic pleuroparenchymal fibroelastosis (PPFE) is a distinctive interstitial pneumonia with upper lobe predominance that shows unique morphological features among idiopathic interstitial pneumonias (IIPs). Affected patients have a variety of clinical presentations with heterogeneous clinical courses. Cluster analysis is a valuable tool for identifying distinct clinical phenotypes under heterogeneous conditions. This study aimed to identify the phenotypes of patients with idiopathic PPFE. Using cluster analysis, novel PPFE phenotypes were identified among subjects from our multicenter cohort, and outcomes were stratified according to phenotypic clusters. Among the subjects with baseline data (N = 84), four clusters were identified. Cluster 1 included younger male subjects with coexisting non-UIP-like patterns. Cluster 2 included elderly female nonsmokers with low body mass index (BMI). Cluster 3 included elderly male smokers with a coexisting IP-like pattern. Cluster 4 included younger male smokers without lower lobe lesions. Patients in cluster 3 had significantly worse survival outcomes than those in clusters 1, 2, and 4 (p < 0.001, p = 0.0041, and p = 0.0155, respectively). Among idiopathic PPFE patients, cluster analysis using baseline characteristics identified four distinct clinical phenotypes that might predict survival outcomes. Full article
(This article belongs to the Special Issue Pleuroparenchymal Fibroelastosis: Obstacles and Challenges)
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Article
Lung Ultrasound to Phenotype Chronic Lung Allograft Dysfunction in Lung Transplant Recipients. A Prospective Observational Study
J. Clin. Med. 2021, 10(5), 1078; https://doi.org/10.3390/jcm10051078 - 05 Mar 2021
Cited by 3 | Viewed by 1144
Abstract
Background: Bronchiolitis obliterans syndrome (BOS) and restrictive allograft syndrome (RAS) are two distinct phenotypes of chronic lung allograft dysfunction (CLAD) in lung transplant (LTx) recipients. Contrary to BOS, RAS can radiologically present with a pleuroparenchymal fibroelastosis (PPFE) pattern. This study investigates lung ultrasound [...] Read more.
Background: Bronchiolitis obliterans syndrome (BOS) and restrictive allograft syndrome (RAS) are two distinct phenotypes of chronic lung allograft dysfunction (CLAD) in lung transplant (LTx) recipients. Contrary to BOS, RAS can radiologically present with a pleuroparenchymal fibroelastosis (PPFE) pattern. This study investigates lung ultrasound (LUS) to identify potential surrogate markers of PPFE in order to distinguish CLAD phenotype RAS from BOS. Methods: A prospective cohort study performed at a National Lung Transplantation Center during June 2016 to December 2017. Patients were examined with LUS and high-resolution computed tomography of the thorax (HRCT). Results: Twenty-five CLAD patients (72% males, median age of 54 years) were included, corresponding to 19/6 BOS/RAS patients. LUS-identified pleural thickening was more pronounced in RAS vs. BOS patients (5.6 vs. 2.9 mm) compatible with PPFE on HRCT. LUS-identified pleural thickening as an indicator of PPFE in RAS patients’ upper lobes showed a sensitivity of 100% (95% CI; 54–100%), specificity of 100% (95% CI; 82–100%), PPV of 100% (95% CI; 54–100%), and NPV of 100% (95% CI; 82–100%). Conclusion: Apical pleural thickening detected by LUS and compatible with PPFE on HRCT separates RAS from BOS in patients with CLAD. We propose LUS as a supplementary tool for initial CLAD phenotyping. Full article
(This article belongs to the Special Issue Pleuroparenchymal Fibroelastosis: Obstacles and Challenges)
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Article
Alveolar Epithelial Denudation Is a Major Factor in the Pathogenesis of Pleuroparenchymal Fibroelastosis
J. Clin. Med. 2021, 10(5), 895; https://doi.org/10.3390/jcm10050895 - 24 Feb 2021
Cited by 1 | Viewed by 1296
Abstract
The pathogenesis of pleuroparenchymal fibroelastosis (PPFE), a rare interstitial lung disease, remains unclear. Based on previous reports and our experience, we hypothesized that alveolar epithelial denudation (AED) was involved in the pathogenesis of PPFE. This multicenter retrospective study investigated the percentage of AED [...] Read more.
The pathogenesis of pleuroparenchymal fibroelastosis (PPFE), a rare interstitial lung disease, remains unclear. Based on previous reports and our experience, we hypothesized that alveolar epithelial denudation (AED) was involved in the pathogenesis of PPFE. This multicenter retrospective study investigated the percentage of AED and the features of the denudated areas in 26 PPFE cases, 30 idiopathic pulmonary fibrosis (IPF) cases, and 29 controls. PPFE patients had lower forced vital capacities and higher residual volume/total lung capacities in pulmonary function tests compared to IPF and control patients. Histopathologically, subpleural fibroelastosis was observed in PPFE, and AED was observed in 12.01% of cases in the subpleural or interlobular septa regardless of fibroelastosis. The percentage of AED in the PPFE group was significantly higher than that in the IPF group (6.84%; p = 0.03) and the normal group (1.19%; p < 0.001). In the IPF group, the percentage of AED and the presence of PPFE-like lesions in the upper lobes were examined radiologically, but no correlation was found. We showed that AED frequently occurred in PPFE. AED was less frequent in IPF, which, in combination with imaging data, suggests that PPFE may have a different pathogenesis from IPF. Full article
(This article belongs to the Special Issue Pleuroparenchymal Fibroelastosis: Obstacles and Challenges)
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Article
Comparison of Clinical Characteristics and Outcomes between Idiopathic and Secondary Pleuroparenchymal Fibroelastosis
J. Clin. Med. 2021, 10(4), 846; https://doi.org/10.3390/jcm10040846 - 18 Feb 2021
Cited by 5 | Viewed by 934
Abstract
Background: Pleuroparenchymal fibroelastosis (PPFE) is a unique clinical, radiologic, and histopathologic entity for which several potential etiologies have been reported recently. However, there has been no comprehensive study of secondary PPFE. Objective: Assessment of the clinical characteristics, outcomes, and prognostic factors of secondary [...] Read more.
Background: Pleuroparenchymal fibroelastosis (PPFE) is a unique clinical, radiologic, and histopathologic entity for which several potential etiologies have been reported recently. However, there has been no comprehensive study of secondary PPFE. Objective: Assessment of the clinical characteristics, outcomes, and prognostic factors of secondary and idiopathic PPFE. Methods: We retrospectively reviewed the medical records of consecutive PPFE patients between January 1999 and December 2018. We identified 132 idiopathic PPFE patients and 32 secondary PPFE patients. Results: The incidence of interstitial lung disease (ILD) pattern different from the usual interstitial pneumonia (UIP) pattern in the lower lobes was higher in secondary PPFE patients (38.5%) than in idiopathic PPFE patients (61.5%, p = 0.02). The idiopathic and secondary PPFE groups did not differ significantly in terms of laboratory data, respiratory complications, and survival (median: 5.0 years vs. 4.1 years, p = 0.95). The presence of UIP pattern was independently associated with increased mortality in multivariate analyses in idiopathic PPFE patients, but not in secondary PPFE patients. Conclusions: The frequency and prognostic impact of UIP-pattern ILD differed between idiopathic and secondary PPFE patients. Lung transplantation should be considered in secondary PPFE patients with low diffusing capacity of the lungs for carbon monoxide (DLCO) regardless of lower-lobe ILD pattern. Full article
(This article belongs to the Special Issue Pleuroparenchymal Fibroelastosis: Obstacles and Challenges)
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Article
Physiological Criteria Are Useful for the Diagnosis of Idiopathic Pleuroparenchymal Fibroelastosis
J. Clin. Med. 2020, 9(11), 3761; https://doi.org/10.3390/jcm9113761 - 22 Nov 2020
Cited by 5 | Viewed by 913
Abstract
Background: Diagnostic criteria of idiopathic pleuroparenchymal fibroelastosis (IPPFE) were recently proposed, including physiological criteria of the body mass index (BMI) and percentage of the predicted values of residual volume (RV)/total lung capacity (TLC) (RV/TLC %pred.). The aim of this study was to evaluate [...] Read more.
Background: Diagnostic criteria of idiopathic pleuroparenchymal fibroelastosis (IPPFE) were recently proposed, including physiological criteria of the body mass index (BMI) and percentage of the predicted values of residual volume (RV)/total lung capacity (TLC) (RV/TLC %pred.). The aim of this study was to evaluate (i) whether the physiologic criteria are useful for the diagnosis and (ii) whether the flat chest index, defined as the ratio of the anteroposterior diameter to the transverse diameter of the thoracic cage, could be an alternative parameter to RV/TLC %pred. Methods: We selected consecutive IPPFE patients and idiopathic pulmonary fibrosis (IPF) patients. We examined the diagnostic sensitivity and specificity of the physiological criteria and flat chest index for differentiating IPPFE patients from IPF patients. Results: This study included 37 IPPFE patients and 89 IPF patients. The physiological criteria distinguished IPPFE patients from IPF patients with a sensitivity of 78.6% and specificity of 88.0%. The combination of the flat chest index and BMI was also effective for differentiation (sensitivity of 82.1% and specificity of 89.3%). Conclusion: We verified the good performance of the physiologic criteria in a different cohort. When the RV/TLC is not measured, using the flat chest index instead of RV/TLC %pred. may be reasonable. Full article
(This article belongs to the Special Issue Pleuroparenchymal Fibroelastosis: Obstacles and Challenges)
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Review

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Review
Lung Transplantation for Pleuroparenchymal Fibroelastosis
J. Clin. Med. 2021, 10(5), 957; https://doi.org/10.3390/jcm10050957 - 01 Mar 2021
Cited by 4 | Viewed by 1188
Abstract
Pleuroparenchymal fibroelastosis (PPFE), a new disease entity associated with interstitial pneumonia, is characterized by fibrosis and elastosis involving the pleura and subpleural lung parenchyma, predominantly in the upper lobe. As the awareness of this disease entity has increased, many studies have revealed the [...] Read more.
Pleuroparenchymal fibroelastosis (PPFE), a new disease entity associated with interstitial pneumonia, is characterized by fibrosis and elastosis involving the pleura and subpleural lung parenchyma, predominantly in the upper lobe. As the awareness of this disease entity has increased, many studies have revealed the prevalence and incidence, clinical and pathological characteristics, and disease course of PPFE. Patients with PPFE reportedly have several unique clinical characteristics—including an extremely low body mass index with a slender body and chest wall deformity, known as “flat chest”. As this disease progresses, shrinking of the lungs often causes life-threatening complications, such as pneumothorax, and associated air leak syndrome. Lung transplantation is considered the only effective treatment for patients with advanced PPFE; however, little is known about the influences of the characteristics of PPFE on the outcome of lung transplantation. This review focuses on the unique clinicopathologic characteristics of PPFE and associated outcomes of lung transplantation for these patients. Full article
(This article belongs to the Special Issue Pleuroparenchymal Fibroelastosis: Obstacles and Challenges)
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