Pleuroparenchymal Fibroelastosis Research Articles

Imaging Features of Idiopathic Interstitial Lung Diseases.

Idiopathic interstitial pneumonias (IIPs) are a group of diffuse parenchymal lung diseases of unclear etiology and are distinguished from diffuse parenchymal lung diseases of known cause, such as connective tissue disease-related interstitial lung diseases or hypersensitivity pneumonitis by history, physical exam, imaging, serologic testing, and, when necessary, histopathology. The 2013 American Thoracic Society (ATS)/European Respiratory Society (ERS) guidelines are the most widely accepted classification of IIPs and include the following diagnoses: idiopathic pulmonary fibrosis, idiopathic nonspecific interstitial pneumonia, cryptogenic organizing pneumonia, acute interstitial pneumonia, idiopathic lymphocytic interstitial pneumonia, idiopathic pleuro-parenchymal fibroelastosis, respiratory bronchiolitis-interstitial lung disease, and desquamative interstitial pneumonia. The gold standard for diagnosis of IIP involves multidisciplinary discussion among pulmonologists, radiologists, and pathologists. The focus of this review will be to discuss the imaging features of the most common IIPs and the role of multidisciplinary discussion as the gold standard for diagnosis.

Autopsy findings in a case of idiopathic pleuroparenchymal fibroelastosis: A rare entity with review of literature

Idiopathic pleuroparenchymal fibroelastosis is a rare idiopathic interstitial pneumonia which mainly affects the upper lobe of lungs resulting in intra alveolar fibrosis, elastosis and visceral pleural fibrosis. For definite diagnosis, radiographic findings as well as histopathology findings are required. The overlap with interstitial lung diseases seen usually, makes it challenging to diagnose. There are no guidelines available for the diagnosis and appropriate management of this disease and only a few cases reported in the literature. We report a case of pleuroparenchymal fibroelastosis in autopsy in a 32-year-old female who allegedly died of heart failure with review of literature.

Lung Transplantation for Pleuroparenchymal Fibroelastosis: A Single-Center Experience with Revision of Literature

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.

Pleuroparenchymal Fibroelastosis-like Lesions in Clinical Practice: A Rare Entity? Review of a Radiological Database.

Background: Pleuroparenchymal Fibroelastosis (PPFE) is a rare disease that consists of elastofibrosis that involves the pleura and subpleural lung parenchyma; it is an unusual pulmonary disease with unique clinical, radiological and pathological characteristics. According to recent studies, PPFE may not be a definite disease but a form of chronic lung injury. The aim of this retrospective study is to determine the incidence and to evaluate the distribution, severity and progression of this radiological entity on high-resolution CT (HRCT) exams of the chest, performed in routine clinical practice. In total, 1514 HRCT exams performed in the period January 2016-June 2018 were analyzed. For each exam, the presence of PPFE was evaluated and a quantitative score was assigned (from 0 to 7 points, based on the maximum depth of fibrotic involvement of the parenchyma). When available, two exams with a time interval of at least 6 months were compared for each patient in order to evaluate progression (defined as the increase in the disease score). Patients were divided into different groups according to exposure and their associated diseases. Statistical analysis was performed by using the Wilcoxon test and Kruskal-Wallis test. Results: PPFE was detected in 174 out of 1514 patients (11.6%), with a mean score of 6.1 ± 3.9 (range 1-14). In 106 out of 174 patients (60.9%), a previous CT scan was available and an evolution of PPFE was detected in 19 of these (11.5%). Among these 19 patients with worsening PPFE, 4 had isolated PPFE that was associated with chronic exposure or connective tissue disorders, and the other 15 had an associated lung disease and/or a chronic exposure. In this group, it was found that the ventral segments of the upper lobes, fissures and apical segments of the lower lobes had a greater statistically significant involvement in the progression of the disease compared to the non-progressive group. In 16 of 174 patients (9.2%, 7 of which belonged to the radiological progression group) a biopsy through video-assisted thoracoscopic surgery or apicoectomy confirmed PPFE. Conclusion: PPFE-like lesions are not uncommon on HRCT exams in routine clinical practice, and are frequently found in patients with different forms of chronic lung injury. Further studies are necessary to explain why the disease progresses in some cases, while in most, it remains stationary over time.

Patterns of Interstitial Lung Disease in Egyptian Patients with Systemic Sclerosis: Relation to Disease Parameters.

Pulmonary involvement is the most common leading cause of morbidity and mortality associated with systemic sclerosis. Therefore, identifying the various patterns of pulmonary affection is crucial in the clinical management of these patients. In the current study, we aim to investigate the patterns of interstitial lung disease (ILD) associated with SSc patients (SSc- ILD) and their relation to serologic markers and clinical parameters. A cross-sectional study was undertaken on thirty-four adult SSc patients who met the 2013 ACR/EULAR criteria for SSc and Forty healthy controls of matched age and sex. The patients were subjected to history taking, clinical examination, skin assessment using the modified Rodnan Skin Score (mRSS), chest x-ray (CXR), pulmonary function test (PFTs), and high resolution computed tomography of the chest (HRCT). Routine laboratory tests were conducted in addition to immunologic tests and an enzyme-linked immunosorbent assay (ELISA) to determine the IL-33 level. ILD was found in 23 SSc patients (67.6%); 20 patients had diffuse type while 3 patients had limited type. Non-specific interstitial pneumonia (NSIP) was found in 56.5%, usual interstitial pneumonia (UIP) was found in 21.7%, pleuroparenchymal fibroelastosis (PPFE) was found in 8.7%, and organizing pneumonia (OP) with the mixed pattern was found in 13% of SSc patients. Additionally, the mean IL-33 level in SSc patients was 98±12.7 compared to 66.2±10.6 in the control group (p < 0.001), with ILD patients having a significantly higher level (101.7±13.4) than those without (90.4±6.2), and a strong positive correlation with mRSS. Even in asymptomatic patients with SSc, ILD is prevalent, with NSIP being the most common pattern. IL-33 could be considered a potential biomarker for predicting the presence of ILD in SSc patients.

Partial pressure of carbon dioxide levels reflect disease severity in idiopathic pleuroparenchymal fibroelastosis

BackgroundHypercapnia can cause a disturbance of consciousness and adversely affect a patient's general condition. Patients with interstitial lung disease seldom experience hypercapnia. Hypercapnia is a typical phenomenon in patients with pleuroparenchymal fibroelastosis (PPFE), especially in advanced stages. However, the clinical significance of hypercapnia in patients with idiopathic PPFE (iPPFE) has not been studied in detail. MethodsWe retrospectively selected patients with iPPFE who had undergone blood gas analysis. The first blood gas data obtained after iPPFE diagnosis were examined. The partial pressure of carbon dioxide (PCO2) levels and their association with characteristic iPPFE parameters, including the flat chest index (the ratio of the anteroposterior diameter of the thoracic cage to the transverse diameter of the thoracic cage), were investigated. ResultsA total of 47 patients with iPPFE were included in this study. The PCO2 level was moderately and inversely correlated with the forced vital capacity. (r = −0.431, P = 0.014), flat chest index (r = −0.497, P < 0.001), and body mass index (r = −0.313, P = 0.038) and was positively correlated with residual volume/total lung capacity. (r = 0.514, P < 0.01). A higher PCO2 level was also significantly associated with poorer prognosis in patients with iPPFE. ConclusionsPCO2 levels could be used as an indicator of disease severity in patients with iPPFE.

Assessment of malnutrition-related risk in patients with idiopathic pleuroparenchymal fibroelastosis.

Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is characterised by upper lobe-dominant fibrosis involving the pleura and subpleural lung parenchyma, with advanced cases often complicated by progressive weight loss. Therefore, we hypothesised that nutritional status is associated with mortality in IPPFE. This retrospective study assesses nutritional status at the time of diagnosis and 1 year after diagnosis in 131 patients with IPPFE. Malnutrition-related risk was evaluated using the Geriatric Nutritional Risk Index (GNRI). Of the 131 patients, 96 (73.8%) were at malnutrition-related risk at the time of diagnosis according to the GNRI. Of these, 21 patients (16.0%) were classified as at major malnutrition-related risk (GNRI <82). Patients at major malnutrition-related risk were significantly older and had worse pulmonary function than patients at low (GNRI 92- <98) and moderate (GNRI 82- <92) malnutrition-related risk. GNRI scores decreased significantly from the time of diagnosis to 1 year after diagnosis. Patients with a lower GNRI (<91.8) had significantly shorter survival than patients with a median GNRI or higher (≥91.8). Patients with declines in annual GNRI scores of ≥5 had significantly shorter survival than patients with declines in annual GNRI scores of <5. In multivariate analysis, major malnutrition-related risk was significantly associated with increased mortality after adjustment for age, sex and forced vital capacity (hazard ratio 1.957). A composite scoring model including age, sex and major malnutrition-related risk was able to separate mortality risk in IPPFE. Assessment of nutritional status by the GNRI provides useful information for managing patients with IPPFE by predicting mortality risk.

Pleuroparenchymal Fibroelastosis: A Review with a Focus on a Non-Infectious Complications after Hematopoietic Stem Cell Transplant

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.

Clinical features of idiopathic pleuroparenchymal fibroelastosis with progressive phenotype showing a decline in forced vital capacity

BackgroundIdiopathic pleuroparenchymal fibroelastosis (IPPFE) is heterogeneous, with some patients showing a progressive decline in forced vital capacity (FVC). However, the clinical features of these cases with progressive phenotypes remain unknown. MethodsThis retrospective study included 48 patients diagnosed with IPPFE who underwent longitudinal pulmonary function tests at our institution from 2005 to 2021. The progressive phenotype was defined as a relative decline of ≥10% in %FVC within two years from diagnosis of IPPFE, and its clinical features were evaluated. ResultsOf the 48 patients, 23 (47.9%) were classified as progressive IPPFE. They were significantly older with a higher rate of dyspnea, fine crackles on chest auscultation, lower-lobe usual interstitial pneumonia (UIP) pattern on high-resolution computed tomography, and lower %FVC at diagnosis than non-progressive IPPFE. Additionally, progressive IPPFE had a significantly higher rate of long-term oxygen therapy requirement, the incidence of pneumothorax, and weight loss after diagnosis, which showed worse survival than non-progressive IPPFE. The relative decline in %FVC and weight loss showed a significant positive correlation. Multivariate analysis revealed that lower body mass index tended to predict early progression, and the coexistence of lower-lobe UIP pattern was significantly associated with early progression. A decline in %FVC was an independent poor prognostic factor in IPPFE. ConclusionsWith a progressive decline in %FVC, IPPFE often has an advanced stage at diagnosis and lower-lobe UIP pattern and is associated with weight loss and worse survival.

Delineating associations of progressive pleuroparenchymal fibroelastosis in patients with pulmonary fibrosis.

Computer quantification of baseline computed tomography (CT) radiological pleuroparenchymal fibroelastosis (PPFE) associates with mortality in idiopathic pulmonary fibrosis (IPF). We examined mortality associations of longitudinal change in computer-quantified PPFE-like lesions in IPF and fibrotic hypersensitivity pneumonitis (FHP). Two CT scans 6-36 months apart were retrospectively examined in one IPF (n=414) and one FHP population (n=98). Annualised change in computerised upper-zone pleural surface area comprising radiological PPFE-like lesions (Δ-PPFE) was calculated. Δ-PPFE >1.25% defined progressive PPFE above scan noise. Mixed-effects models evaluated Δ-PPFE against change in visual CT interstitial lung disease (ILD) extent and annualised forced vital capacity (FVC) decline. Multivariable models were adjusted for age, sex, smoking history, baseline emphysema presence, antifibrotic use and diffusion capacity of the lung for carbon monoxide. Mortality analyses further adjusted for baseline presence of clinically important PPFE-like lesions and ILD change. Δ-PPFE associated weakly with ILD and FVC change. 22-26% of IPF and FHP cohorts demonstrated progressive PPFE-like lesions which independently associated with mortality in the IPF cohort (hazard ratio 1.25, 95% CI 1.16-1.34, p<0.0001) and the FHP cohort (hazard ratio 1.16, 95% CI 1.00-1.35, p=0.045). Progression of PPFE-like lesions independently associates with mortality in IPF and FHP but does not associate strongly with measures of fibrosis progression.

Immune Checkpoint Inhibitor-Related Pneumonitis in Lung Cancer Patients with Interstitial Lung Disease: Significance of Radiological Pleuroparenchymal Fibroelastosis

Introduction: Pleuroparenchymal fibroelastosis (PPFE) findings are associated with poor prognosis in interstitial lung disease (ILD). However, the effect of PPFE findings on the development of immune checkpoint inhibitor-related pneumonitis (ICI-pneumonitis), a life-threatening adverse event, in lung cancer patients with ILD has not been elucidated. We aimed to determine whether PPFE findings are a risk factor for ICI-pneumonitis in lung cancer patients with ILD. Methods: We retrospectively examined 712 lung cancer patients, including 173 patients with background ILDs, who received ICI therapy in our institute between December 2015 and May 2021. Background ILDs were radiologically classified into three types: lone PPFE, other ILDs with PPFE, and other ILDs without PPFE. The cumulative ICI-pneumonitis incidence curves and median overall survival (mOS) were compared between the three radiological types, and risk factors for ICI-pneumonitis were evaluated. Results: Of 173 eligible patients with ILD, 23 patients (13.3%) experienced ICI-pneumonitis. The Kaplan-Meier method and the log-rank test showed that lone PPFE patients had significantly lower incidence of ICI-pneumonitis (p = 0.024) and longer mOS (575 vs. 326 days; p = 0.0096) than other ILDs patients. ICI-pneumonitis (p = 0.35) and mOS (p = 0.29) were not significantly different between other ILDs with and without PPFE. A multivariate Cox proportional hazards regression analysis revealed that lone PPFE pattern was an independent predictive factor for ICI-pneumonitis (hazard ratio, 0.20; 95% confidence interval, 0.043–0.93; p = 0.040). Conclusion: ICI therapy could be safer in lone PPFE patients than in other ILDs patients with lung cancer.

A Rare Manifestation of Asbestos Induced Lung Disease

Pleuroparenchymal fibroelastosis (PPFE) is a rare pulmonary condition that is often clinically characterised by lung upper-lobe predominant fibrosis. This article describes a case of a gentleman with shortness of breath over years who has subsequently been diagnosed with PPFE.

ОПЫТ ВЕДЕНИЯ ПАЦИЕНТКИ С ИДИОПАТИЧЕСКИМ ПЛЕВРОПАРЕНХИМАТОЗНЫМ ФИБРОЭЛАСТОЗОМ: ОСОБЕННОСТИ ДИАГНОСТИКИ И ЛЕЧЕНИЯ

Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a rare form of idiopathic interstitial pneumonia the etio¬logy of which is often unknown, and the course of the disease is usually rapidly progressive. The disease occurs with equal frequency in both men and women. Its onset usually is observed in the second half of life. Complaints are nonspecific: patients suffer from dry cough, shortness of breath, low body mass. A distinctive feature of IPPFE is the involvement of the apices of the lungs and development of platythorax, which can be easily detected in these patients during computed tomography. There is no specific treatment, but there is an evidence of positive experience with the use of antifibrotic drugs.

Clinical Diagnosis of Pleuroparenchymal Fibroelastosis

Pleuroparenchymal fibroelastosis (PPFE) is an extremely rare interstitial lung disease (ILD) characterised by fibroelastotic changes predominating in the subpleural lung parenchyma with visceral pleural fibrosis involving the upper lobes. It has distinctive clinical, radiological, and pathological manifestations. Diagnosis constitutes literally a stalemate because of its rarity, unavailability of an agreed diagnostic consensus, and requirement of tissue biopsy for an accurate final diagnosis that can not be performed in most of the patients owing to the comorbid complications of the disease itself. Identification of PPFE may also pose great difficulties due to the coexistence of other interstitial lung diseases. PPFE usually exhibits a persistently deteriorating prognostic course culminating in fatal complications including respiratory failure, pulmonary hypertension, cor pulmonale, or pneumothorax. Presence of disease relevant complications frequently preclude invasive tissue biopsy interventions in these patients leading to a diagnostic challenge for clinicians. This review aims to provide a definitive diagnosis based on entirely the clinical manifestatins of PPFE by shedding light on the pathogenesis, clinical, and radiologic findings of the disease. Histopathological tissue evaluation was also included in the diagnostic approach for patients whose condition was suitable for an invasive biopsy intervention. With this review, it is concluded that an approach consisting of exclusively clinical manifestations will ensure adequate support for an accurate PPFE diagnosis without any requirement for histopathological tissue examination.

HPR30 Too Ultra-Rare for Care? Orphan Drug Availability for People Living With Ultra-Rare Respiratory Diseases in the Pre, Present and Post Pandemic Worlds

To compare the current and potential availability of treatments for seven index ultra-rare respiratory diseases before and after the peak of the COVID-19 pandemic. At the 2019 ISPOR EU Conference, a landscape review of available treatments for ultra-rare respiratory diseases was presented. Therefore, 3 years on, we sought to explore if treatment access has improved for ultra-rare respiratory diseases. A landscape review was undertaken to seek evidence of treatment developments for ataxia telangiectasia (AT), lymphangiomatosis (LYMF), pulmonary alveolar proteinosis (PAP), pleuroparenchymal fibroelastosis (PPFE), pulmonary alveolar microlithiasis (PAM), pulmonary dendriform ossification (PDO), and light chain deposition disorders (LCDD). Information from clinicaltrials.gov, orpha.net, the EMA and FDA archives and websites of five health technology assessment (HTA) bodies was narratively synthesised. The 2019 clinicaltrials.gov search identified 24 studies and the 2022 search 25 studies at various stages of the clinical trial process. They concerned PAP (2019/2022: 11/10), AT (6/10), LCDD (5/4), LYMF (1/0) and PPFE (1/1). No studies were identified for PAM or PDO. The 2019 review found treatments for AT and PAP were granted orphan status by the EMA and the FDA, and in 2021 the FDA granted orphan status to another AT treatment. Neither search found reimbursement submissions for any of the ultra-rare respiratory disease. There still remains an obvious lack of proven and available treatments for ultra-rare respiratory diseases. Over the past 3 years, only 1 new treatment has been granted regulatory approval and no treatments have yet to result in therapies that are licensed or approved by HTA bodies. It is our hope that as we enter the post-pandemic world, we as industry professionals and researchers start to adequately address the treatment needs of people living with these debilitating conditions.

Idiopathic pleuroparenchymal fibroelastosis

The term idiopathic pleuroparenchymal fibroelastosis refers to a rare interstitial lung disease that predominantly involves the upper lobes. It has been considered a rare subtype of interstitial lung disease since 2013, when it was included in the joint consensus statement on the diagnosis of interstitial lung diseases published by the American Thoracic Society (ATS) and the European Respiratory Society (ERS).Currently, two distinct types of pleuroparenchymal fibroelastosis are recognized: the idiopathic type for cases in which it has not been possible to establish a specific etiology and a secondary type associated with a variety of different causes.The diagnosis of pleuroparenchymal fibroelastosis must be managed from a combined clinical and radiological perspective. High-resolution computed tomography (HRCT) is the imaging method of choice for the evaluation and diagnosis of pleuroparenchymal fibroelastosis. In many cases, the diagnosis will be based exclusively on the HRCT findings and histologic confirmation will be unnecessary.This article describes the clinical, radiological, and histological characteristics of pleuroparenchymal fibroelastosis, discussing the different associations with this entity and its differential diagnosis.

A stepwise multi-disciplinary algorithm for diagnosis of fibrosing lung diseases contributing MDCT, MRI, and PET/CT: a study on 250 patients using significance and validation analyses

BackgroundThe new guidelines limited the use of lung biopsy in the evaluation of lung fibrosis because of its hazards. The differential diagnosis of interstitial pulmonary fibrosis (IPF) or usual interstitial pneumonia (UIP) is challenging because of overlapping multi-detector computed tomography (MDCT) morphologic features between interstitial and non-interstitial fibrosing lung diseases. Scar carcinoma is a serious complication that needs to be excluded in certain conditions. Aim of the work: To achieve a multi-disciplinary algorithm for the diagnosis of fibrosing lung diseases to limit the need for lung biopsy by combining the clinico-laboratory and radiological roles.ResultsThis study included two major steps. The first step (prevalence/significance analysis of the contributing parameters for the diagnosis of fibrosing lung diseases) was retrospectively conducted on 150 patients pathologically proved with fibrosing lung disease during the period between January/2016 and April/2018. Based on a P-value < 0.001, honeycombing bronchiectasis was significant to IPF. Basal traction bronchiectasis/bronchiolectasis was relevant to fibrosing non-specific interstitial pneumonia (NSIP). "Head cheese" CT-sign, history of allergen exposure, blood eosinophilia, and broncho-alveolar lavage (BAL) lymphocytosis were relevant to chronic hypersensitivity pneumonitis (HP). Upper peripheral lung fibrosis was significant to pulmonary tuberculosis (TB) and pleuroparenchymal fibroelastosis (PPFE). Cavitations, tree-in-bud, and calcific nodules were relevant to TB, while the "platy-thorax" CT-sign was relevant to PPFE. The upper peribronchovascular fibrosis was relevant to sarcoidosis and progressive massive fibrosis (PMF); additionally, calcific changes were relevant to PMF. Bright T2-signal, diffusion weighted-image (DWI) restriction in magnetic-resonance imaging (MRI), and high standardized uptake value (SUV) in positron emission tomography (PET-CT) were significant to scar carcinoma. Eventually, an algorithm was created. The second step (validation analysis) prospectively targeted 100 patients initially diagnosed with lung fibrosis during the period from June/2018 to June/2022. It revealed 83.3–100% sensitivity, 96.3–100% specificity, 85.7–100% PPV, 96.4–100% NPV, and 96–100% accuracy, with balanced accuracy = 0.91–1. Four consulting radiologists and two consulting pulmonologists participated in this study.ConclusionsA valid stepwise multi-disciplinary algorithm was proposed for the diagnosis of interstitial and non-interstitial fibrosing lung diseases to limit the need and hazards of lung biopsy. It contributed significant clinico-laboratory data, MDCT features, T2-WI and DWI-MRI findings as well as PET/CT results.

Bilateral pneumothoraces with apical consolidations.

Bilateral pneumothoraces are extremely rare. Patients with pleuroparenchymal fibroelastosis are frequently complicated by pneumothorax, which is sometimes bilateral. Pleuroparenchymal fibroelastosis should be considered a cause of bilateral pneumothoraces with apical fibrosis.

Prognostic significance of radiological pleuroparenchymal fibroelastosis in Mycobacterium avium complex lung disease: a multicentre retrospective cohort study

BackgroundMycobacterium avium complex (MAC) causes chronic respiratory infectious diseases with diverse clinical features and prognoses. Pleuroparenchymal fibroelastosis (PPFE) is a rare disease characterised by pleural fibrosis with subjacent intra-alveolar fibrosis...

Fibroelastosis pleuroparenquimatosa idiopática (FEPPI)

The term idiopathic pleuroparenchymal fibroelastosis refers to a rare interstitial lung disease that predominantly involves the upper lobes. It has been considered a rare subtype of interstitial lung disease since 2013, when it was included in the joint consensus statement on the diagnosis of interstitial lung diseases published by the American Thoracic Society (ATS) and the European Respiratory Society (ERS).Currently, two distinct types of pleuroparenchymal fibroelastosis are recognized: the idiopathic type for cases in which it has not been possible to establish a specific etiology and a secondary type associated with a variety of different causes.The diagnosis of pleuroparenchymal fibroelastosis must be managed from a combined clinical and radiological perspective. High-resolution computed tomography (HRCT) is the imaging method of choice for the evaluation and diagnosis of pleuroparenchymal fibroelastosis. In many cases, the diagnosis will be based exclusively on the HRCT findings and histologic confirmation will be unnecessary.This article describes the clinical, radiological, and histological characteristics of pleuroparenchymal fibroelastosis, discussing the different associations with this entity and its differential diagnosis.