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Biomedicines
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Interstitial Lung Diseases: Focus on Pleuroparenchymal Fibroelastosis
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Interstitial Lung Diseases: Focus on Pleuroparenchymal Fibroelastosis

A special issue of Biomedicines (ISSN 2227-9059). This special issue belongs to the section "Molecular and Translational Medicine".

Deadline for manuscript submissions: closed (30 June 2023) | Viewed by 5283

Special Issue Editor

Dr. Martina Bonifazi

E-Mail Website
Guest Editor
Department of Biomedical Sciences and Public Health, Università Politecnica delle Marche, Ancona, Italy
Interests: interstitial lung diseases; interventional pulmonology; pleural diseases; chronic obstructive lung diseases; epidemiology

Special Issue Information

Dear Colleagues,

The term "interstitial lung diseases" (ILDs) includes a wide spectrum of heterogeneous entities with different prognoses and treatment options. Pleuroparenchymal fibroelastosis (PPFE) is a specific clinicopathologic entity that affects the visceral pleura and the subpleural parenchyma with an upper-lobe predilection, characterized by elastic intra-alveolar fibrosis and scattered fibroblastic foci. Its pathogenesis is still unclear, but the heterogeneous spectrum of clinical presentations and behavior suggests that it may represent the final expression of a variable interplay between immune dysregulation, environmental exposure, and genetic predisposition. PPFE can present as an idiopathic form or in association with a variety of different conditions, including infections, lung and bone marrow transplantation, and autoimmune diseases. Moreover, PPFE features have been reported in association with other interstitial lung diseases, including idiopathic pulmonary fibrosis (IPF), hypersensitivity pneumonitis (HP), and familial forms of pulmonary fibrosis. The increasing awareness of PPFE among specialists over recent years has led to an increase in its identification in both idiopathic and secondary contexts, suggesting that it may not be as rare as previously thought. However, to date, there is no consensus on optimal diagnostic strategy nor curative are treatments available yet. The main goals of this Special Issue are to update and summarize insights and challenges of PPFE, and leaders in the different fields will be invited to provide comprehensive reviews on interstitial lung disease topics. 

Dr. Martina Bonifazi
Guest Editor

Manuscript Submission Information

Manuscripts should be submitted online at www.mdpi.com by registering and logging in to this website. Once you are registered, click here to go to the submission form. Manuscripts can be submitted until the deadline. All submissions that pass pre-check are peer-reviewed. Accepted papers will be published continuously in the journal (as soon as accepted) and will be listed together on the special issue website. Research articles, review articles as well as short communications are invited. For planned papers, a title and short abstract (about 100 words) can be sent to the Editorial Office for announcement on this website.

Submitted manuscripts should not have been published previously, nor be under consideration for publication elsewhere (except conference proceedings papers). All manuscripts are thoroughly refereed through a single-blind peer-review process. A guide for authors and other relevant information for submission of manuscripts is available on the Instructions for Authors page. Biomedicines is an international peer-reviewed open access monthly journal published by MDPI.

Please visit the Instructions for Authors page before submitting a manuscript. The Article Processing Charge (APC) for publication in this open access journal is 2600 CHF (Swiss Francs). Submitted papers should be well formatted and use good English. Authors may use MDPI's English editing service prior to publication or during author revisions.

Keywords

  • interstitial lung diseases
  • pleuroparenchymal fibroelastosis
  • idiopathic pulmonary fibrosis
  • hypersensitivity pneumonitis
  • diagnosis

Published Papers (3 papers)

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Research

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10 pages, 1428 KiB  
Article
Lung Transplantation for Pleuroparenchymal Fibroelastosis: A Single-Center Experience with Revision of Literature
by Eleonora Faccioli, Vincenzo Verzeletti, Chiara Giraudo, Marco Schiavon, Fiorella Calabrese, Monica Loy, Federico Rea and Andrea Dell’Amore
Biomedicines 2023, 11(6), 1505; https://doi.org/10.3390/biomedicines11061505 - 23 May 2023
Cited by 1 | Viewed by 980
Abstract
Pleuroparenchymal fibroelastosis (PPFE) is a rare condition characterized by fibrosis involving the pleura and the upper lobes which can be idiopathic or secondary to chemotherapy, transplantations and occupational exposure. For the end-stage form, lung transplantation (LT) is the treatment of choice. The aim [...] Read more.
Pleuroparenchymal fibroelastosis (PPFE) is a rare condition characterized by fibrosis involving the pleura and the upper lobes which can be idiopathic or secondary to chemotherapy, transplantations and occupational exposure. For the end-stage form, lung transplantation (LT) is the treatment of choice. The aim of this study was to report our single-center experience for patients subjected to LT for PPFE and comparing it with the already published evidence on this topic. At our center, we have performed 6 bilateral LTs for patients with PPFE (3 males and 3 females) with a median age of 52 years. Median ICU and in-hospital length of stay were 8 and 30 days, respectively. To date, two patients are alive and four are dead, with a median overall survival of 10 months. In addition, after a formal search using the terms “pleuroparenchymal fibroelastosis AND lung transplantation”, we collected 14 studies focused on outcomes after LT. LT for PPFE is technically challenging and its post-operative course could also be complicated. Current available data on LT outcomes are extremely poor and mostly limited to case reports. Further studies need to be published to improve knowledge of this disease and to achieve best outcomes for LT. Full article
(This article belongs to the Special Issue Interstitial Lung Diseases: Focus on Pleuroparenchymal Fibroelastosis)
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10 pages, 453 KiB  
Article
Determinants of Six-Minute Walk Distance in Idiopathic Pulmonary Fibrosis and Idiopathic Pleuroparenchymal Fibroelastosis
by Naofumi Sato, Yuji Iwanami, Kento Ebihara, Keiko Nakao, Midori Miyagi, Yasuhiko Nakamura, Kazuma Kishi, Sakae Homma and Satoru Ebihara
Biomedicines 2022, 10(10), 2556; https://doi.org/10.3390/biomedicines10102556 - 13 Oct 2022
Cited by 3 | Viewed by 1831
Abstract
Background: In idiopathic pulmonary fibrosis (IPF), 6-minute walking distance (6MWD) is an independent factor for mortality. Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a rare disease with physical features such as emaciation, but the relationship between IPPFE and 6MWD is unclear. In this study, we [...] Read more.
Background: In idiopathic pulmonary fibrosis (IPF), 6-minute walking distance (6MWD) is an independent factor for mortality. Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a rare disease with physical features such as emaciation, but the relationship between IPPFE and 6MWD is unclear. In this study, we investigated the factors that cause a decrease in the percent of the predicted value of a 6-minute walk distance (%6MWD), including the disease entities, IPF and IPPFE. Methods: This study included 100 patients (IPF: 75 patients, IPPFE: 25 patients, age: 73.5 ± 7.2 years, sex: 73 males) who visited the rehabilitation department. Patients with a %6MWD ≥ 80% were assigned to the normal group (n = 54), and patients with a %6MWD < 80% were assigned to the decreased group (n = 46). The items showing a significant difference between groups were used as independent variables, after the consideration of multicollinearity, for a logistic analysis where %6MWD < 80% was used as the dependent variable. Results: The 6MWD results show that there was no significant difference between IPF and IPPFE in the absolute value of 6MWD and in the number of people with 6MWD ≥ 250 m, but when 6MWD was compared with %6MWD, the IPPFE group showed a significantly lower value than the IPF group (p = 0.013). Logistic regression analysis showed that only BMI (p = 0.032), GAP index (p = 0.043), and mMRC (p = 0.026) were factors that caused a decrease in %6MWD in 100 patients. Conclusion: The results suggest that leanness, shortness of breath and severity of illness are the most important factors that determine exercise tolerance, regardless of disease entity in IPF and IPPFE. Full article
(This article belongs to the Special Issue Interstitial Lung Diseases: Focus on Pleuroparenchymal Fibroelastosis)
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Review

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11 pages, 270 KiB  
Review
Pleuroparenchymal Fibroelastosis: A Review with a Focus on a Non-Infectious Complications after Hematopoietic Stem Cell Transplant
by Patrick Arndt
Biomedicines 2023, 11(3), 924; https://doi.org/10.3390/biomedicines11030924 - 16 Mar 2023
Cited by 1 | Viewed by 1993
Abstract
Pleuroparenchymal fibroelastosis (PPFE) is a rare disease that is currently classified as an idiopathic interstitial pneumonia. Although originally described as an idiopathic disease, PPFE has now been identified as a rare complication following hematopoietic stem cell transplant (HSCT). Unlike other pulmonary complications after [...] Read more.
Pleuroparenchymal fibroelastosis (PPFE) is a rare disease that is currently classified as an idiopathic interstitial pneumonia. Although originally described as an idiopathic disease, PPFE has now been identified as a rare complication following hematopoietic stem cell transplant (HSCT). Unlike other pulmonary complications after HSCT, PPFE occurs very late after transplant. Etiologies for PPFE after HSCT remain to be fully established. Infections and adverse effects to alkylating chemotherapy have been suggested as possible causes. In several cases, there is an association of PPFE with bronchiolitis obliterans syndrome after HSCT, suggesting that PPFE may be another manifestation of pulmonary chronic graft versus host disease after HSCT. Algorithms have been designed to assist in confirming a diagnosis of PPFE without the need for a surgical lung biopsy, however at present, no biomarker is established for the diagnosis or to predict the progression of disease. Presently, there is no current therapy for PPFE, but fortunately the disease progresses slowly in most patients. Full article
(This article belongs to the Special Issue Interstitial Lung Diseases: Focus on Pleuroparenchymal Fibroelastosis)
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