Pleuroparenchymal fibroelastosis: is it also an idiopathic entity?

Pleuroparenchymal fibroelastosis (PPFE) is a rare disease that has been recently included in the updated consensus on idiopathic interstitial pneumonias. It shares some clinical features with other chronic interstitial pneumonias (dyspnea, dry cough), and is radiologically characterized by pleural and subpleural parenchymal fibrosis and elastosis, mainly in the upper lobes. The main histological findings include pleural fibrosis and prominent subpleural and parenchymal fibroelastosis. Its characterization is based on the increasing number of cases reported in the literature, so several aspects of the etiology, pathogenesis and natural history are still unknown. Although some cases have been described as idiopathic, PPFE has been reported as a complication after bone marrow transplantation, lung transplantation and chemotherapy, especially with alkylating agents.Spontaneous or iatrogenic pneumothorax is a frequently reported complication of invasive diagnostic tests for identifying PPFE. The disease course is variable, ranging from slow progression to rapid clinical deterioration. No treatment has shown evidence of efficacy, and lung transplantation remains the only option for patients who fulfill the diagnostic criteria for this option. Recognizing and disseminating the specific features of PPFE is essential to raise the level of clinical suspicion for this entity, and to implement appropriate multidisciplinary diagnostic management.

Medical thoracoscopy: a useful diagnostic tool for undiagnosed pleural effusion.

OBJECTIVE: We aimed to assess the role of medical thoracoscopy in patients with undiagnosed pleural effusion. METHODS: Patiens presenting with pleural effusion underwent three pleural aspirations. Patients in whom pleural fluid analysis was inconclusive underwent closed pleural biopsy for diagnostic confirmation. Patients in whom closed pleural biopsy was incolcusive underwent medical thoracoscopy using a rigid thoracoscope with a viewing angle of zero degrees was done under local anaesthesia and sedation with the patient lying in lateral decubitus position with the affected side up. Biopsy specimens from parietal pleura were obtained under direct vision and were sent for histopathological examination. RESULTS: Of the 128 patients with pleural effusion who were studied, pleural fluid examination established the diagnosis in 81 (malignancy 33, tuberculosis 33, pyogenic 14 and fungal 1); 47 patients underwent closed pleural biopsy and a diagnosis was made in 28 patients (malignancy 24, tuberculosis 4). The remaining 19 patients underwent medical thoracoscopy and pleural biopsy and the aetiological diagnosis could be confirmed in 13 of the 19 patients (69%) (adenocarcinoma 10, poorly differentiated carcinoma 2 and mesothelioma 1). CONCLUSION: Medical thoracoscopy is a useful tool for the diagnosis of pleural diseases. The procedure is safe with minimal complications.

Idiopathic pleuroparenchymal fibroelastosis: consideration of a clinicopathological entity in a series of Japanese patients.

BACKGROUND: Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a recently reported group of disorders characterized by fibrotic thickening of the pleural and subpleural parenchyma predominantly in the upper lobes. We report five Japanese cases fulfilling the criteria of IPPFE and address whether it should be considered a separate clinicopathologic entity. And this study was an attempt to identify features in common between IPPFE and previously described idiopathic upper lobe fibrosis (IPUF), allowing IPPFE to be considered as a distinct entity in our Japanese series. METHODS: Five consecutive cases of idiopathic interstitial lung disease confirmed as IPPFE by surgical lung biopsy were studied. RESULTS: There were four males and one female, aged 70±2.76 yr. No associated disorder or presumed cause was found in any case. Lung function tests found a restrictive ventilatory defect (4/5) and/or impairment of DLco (4/5). Chest X-ray showed marked apical pleural thickening in all cases. Computed tomography of the chest in all cases mainly showed intense pleural thickening and volume loss associated with evidence of fibrosis, predominantly in the upper lobes. In all cases in this study, markedly thickened visceral pleura and prominent subpleural fibrosis characterized by both elastic tissue and dense collagen were clearly shown. All cases were alive at the last follow-up, 17.6±13.59 months after diagnosis; however, all had deteriorated both clinically and radiologically. CONCLUSIONS: IPPFE deserves to be defined as a separate, original clinicopathologic entity owing to its uniformity and IPPFE has some features in common with previously described idiopathic upper lobe fibrosis (IPUF). Our limited experience with a cohort of 5 subjects suggests that IPPFE can be rapidly progressive.

Pleural plaque profiles on the chest radiographs and CT scans of asbestos-exposed Japanese construction workers.

Pleural plaques are asymptomatic focal thickenings of the pleura and considered the hallmark of asbestos exposure. However, it is often difficult to detect pleural plaques on chest x-rays (CXR). In a retrospective study, using chest CT scans of 140 Japanese asbestos-exposed construction workers who have probable or definite findings of pleural plaque on CXR; firstly, we proposed plaque morphology-based classification for CXR findings, and then we examined if those classified findings could be confirmed as pleural plaques on CT scans. Our morphology-based classification of pleural plaque findings included nine types. The percentages of confirmed pleural plaques on CT scans by type (number of confirmed pleural plaque on CT/number of observed on CXR) were 93% (40/43) for straight, 89% (56/63) for diamond, 88% (7/8) for double, 83% (19/23) for tapered medially, 80% (20/25) for parallel, 77% (23/30) for crescent, 79% (11/14) for tenting, 72% (18/25) for tapered-laterally (long type), and 0% (0/9) for tapered-laterally (short type). When added to the ILO classification, morphology-based classification of CXR pleural plaque findings makes its detection easier and hence chest radiograph continues to be a suitable tool for screening asbestos-related pleural plaques based on its simplicity, low radiation exposure, wide availability and cost-effectiveness.

Continued exposure to silica after diagnosis of silicosis in Brazilian gold miners.

BACKGROUND: There is a paucity of studies analyzing the effect of continued silica exposure after the onset of silicosis with regard to disease progression. The present study investigates differences in clinical and radiological presentation of silicosis among former workers with a diagnosis of silicosis, and compares workers who continued to be exposed to silica with those who stopped silica exposure after having received their diagnosis. METHODS: A sample of 83 former gold miners with a median of 21 years from the first diagnoses of silicosis, had their clinical and occupational histories taken and underwent both chest radiography (International Labor Organization standards) and spirometry. Their silica exposure was assessed and an exposure index was created. The main outcome was the radiological severity of silicosis and tuberculosis (TB). The statistical analysis was done by multiple logistic regression. RESULTS: Among the 83 miners, 44 had continued exposed to silica after being diagnosed with silicosis. Continuation of silica exposure was associated with advanced radiological images of silicosis (X-ray classification in category 3, OR = 6.42, 95% CI = 1.20-34.27), presence of coalescence and/or large opacities (OR = 3.85, CI = 1.07-13.93), and TB (OR = 4.61, 95% CI = 1.14-18.71). CONCLUSIONS: Differential survival is unlikely to explain observed differences in silicosis progression. Results reinforce the recommendation that silica exposure should be halted at an early stage whenever X-ray is suggestive of the disease.

Urinothorax: a new approach.

PURPOSE OF REVIEW: The presence of urine in the pleural space (urinothorax) is a rarely recognized cause of pleural effusion. To date, only 58 cases have been reported. In this article the features of urinothorax are analyzed, and clinical and biochemical characteristics are reviewed in order to propose a classification, founded on pathogenic criteria, that will be useful in achieving the diagnosis. RECENT FINDINGS: Recently reported cases of urinothorax provide a more detailed description of the biochemical characteristics that allow a better understanding of this entity. SUMMARY: Urinothorax can be divided into two categories: (1) obstructive urinothorax, due to bilateral obstructive uropathy; and (2) traumatic urinothorax, due to unilateral traumatic injury of the urinary system, mostly iatrogenic. In patients with urinothorax, the pleural effusion usually has the biochemical characteristics of a transudate, with a pH lower than 7.30 and a pleural fluid/serum creatinine ratio higher than 1. These characteristics are not always present, however, and individually are shared by a significant number of pleural effusions of different etiology.

Enfermedades pleurales benignas inducidas por asbesto / Benign pleural diseases induced by asbestos

La exposición al asbesto es una causa importantede patología pleural y se puede producir con intensidadesmoderadas o ligeras dada la capacidad delasbesto de concentrarse en la pleura. Ello motiva juntoa la prolongada latencia existente entre la exposición yla enfermedad, que sigamos viendo durante muchosaños manifestaciones clínicas pleurales de exposiciónprevia, a pesar del uso del asbesto cada vez más limitadoen las últimas décadas. Dicha exposición puedepresentarse con distintas manifestaciones tanto malignascomo el mesotelioma como benignas, siendo lasprincipales de éstas el derrame pleural benigno, lasplacas pleurales, la fibrosis pleural difusa y la atelectasiaredonda

Exposure to asbestos is an important cause ofpleural pathology and can be produced with light ormoderate tendencies given the capacity of asbestos toconcentrate in the pleura. Together with the prolongedlatency existing between exposure and the disease,this means that for many years we will continue to seepleural clinical manifestations from past exposure, inspite of the increasingly limited use of asbestos inrecent decades. This exposure can show itself indifferent manifestations, both malign, such asmesothelioma, and benign, principally benign pleuraleffusion, pleural plaques, diffuse pleural fibrosis andmassive atelectasis

Benign pleural diseases.

The pleural space is a potential space under normal physiologic circumstances. It envelops the lung, the mediastinum, the diaphragm and the chest wall. A thin film of pleural fluid provides lubrication for the two pleural layers; only 2-10 ml of pleural fluid is present in healthy people. For the purposes of this review, pleural abnormalities will be divided into pleural effusion, pneumothorax, and pleural calcification.

Observer variation in classifying chest radiographs for small lung opacities and pleural abnormalities in a population sample.

BACKGROUND: The purpose of this study was to assess inter-and intraobserver variation in the radiographic categories of small lung opacities (profusion) and pleural abnormalities classified according to the ILO classification of pneumoconioses with some modifications. METHODS: Chest radiographs derived from a representative adult population sample (n = 7,095) were classified by two radiologists. Observer variation was assessed on the basis of kappa (kappa)-type statistics. RESULTS: The observers agreed on profusion categories in 69% of cases of the total material. Up to 98% of the classifications fell into the same category or deviated by no more than one category. The corresponding kappa (kappa) coefficient was 0.48 (95% CI = 0.46=0.49) and the weighted kappa 0.72. When a selected subsample was reclassified by the observers, the proportions of crude agreement on profusion of small opacities ranged from 42% to 47% (weighted kappa 0.52-0.55). The proportions of agreement on the main pleural abnormalities were 92% or over, and the corresponding kappa coefficients at least 0.73. CONCLUSION: The classification of lung opacities was subject to considerable observer variation, which calls for caution when results from different studies are compared. This variation, however, rarely exceeded one category, and thus appears to be small enough for meaningful comparisons between groups, at least within a single study.

Pleural disease in patients undergoing lung transplantation for cystic fibrosis.

Cystic fibrosis (CF) is associated with varying degrees of pleural inflammatory reaction that occurs as a result of chronic pulmonary infections and intervention to pleural space. The amount of pleural reaction is associated with the difficulty involved when performing the pneumonectomy at the time of lung replacement. The aim of this study is to identify possible pre-transplantation parameters that may predict the extent of pleural adhesion density. The charts of the 32 CF patients who underwent lung transplantation were reviewed. The degree of pleural adhesions was graded as none, minimal, moderate or severe, by extensive retrospective chart review of the operative and pathology reports. Available Brasfield radiographic scores, chest computerized tomographic (CT) scan scores, and pulmonary function test results were correlated to the pleural density grades. CT scans were scored by one radiologist as none, mild focal, moderate focal, diffuse mild or severe pleural disease. The presence of severe adhesions was associated with lower diffusion capacity corrected for volume (DL(CO)/VA) (p=0.0022) and older age (p<0.05) at the time of transplant. The typical radiographic and pulmonary function findings of airway obstruction, parenchymal nodularity and air trapping did not correlate with pleural adhesion density. The eleven patients with severe pleural adhesions had a longer ICU course and remained intubated longer than the thirteen patients with none or minimal adhesions. The extent of severe adhesions associated with CF cannot be predicted by preoperative Brasfield radiographic scores of parenchymal disease or spirometry data. However, chest CT imaging and DL(CO)/VA measurements may be useful in predicting the extent of pleural adhesions and the degree of dissection difficulty associated with the pneumonectomies for lung transplantation in the CF recipients.

Pleuritis inespecífica: etiología y seguimiento

Introducción: En el estudio de algunos derrames pleurales de tipo exudado, la biopsia pleural informa pleuritis inespecífica (PI). No hay criterios que permitan pronosticar cuál será la evolución de estos pacientes ni hay acuerdo acerca de cuál debe ser la conducta ante el informe de PI. Objetivos: Identificar características clínicas, radiológicas o de laboratorio que permitan predecir la evolución de los pacientes con PI y sugieren la enfermedad subyacente. Pacientes y Métodos: Se revisó la historia y la evolución de los pacientes con derrame de tipo exudado que fueron sometidos a una primera biopsia pleural en la cual una muestra de buena calidad informó PI. Se excluyeron aquellos con ADA > 50 U y citología sospechosa o definitiva de malignidad. Se comparó el número de biopsias y procedimientos a que fue sometido cada paciente después de recibir el informe de PI y los diagnósticos finales. Se buscaron diferencias entre grupos de acuerdo con el diagnóstico definitivo (tuberculosis y cáncer, resolución espontánea, otra causa de PI y muerte sin diagnóstico). Resultados: De 430 biopsias tomadas durante el período de estudio (1991-1993), 60 fueron informadas como PI. Se obtuvieron datos completos en 43 casos. Las características clínicas, radiológicas y bioquímicas fueron similares entre los grupos. Solamente la pérdida de peso se asoció con una mayor probabilidad de tuberculosis o cáncer. El uso de toracoscopia se asoció con mayor probabilidad de alcanzar un diagnóstico definitivo. Conclusión: Si se incluye en la definición de PI un nivel de ADA inferior a 50 U (para excluir TBC) y un resultado de la citología que no sugiera cáncer, ningún factro clínico o paraclínico permite pronosticar la presencia de tuberculosis o cáncer en pacientes con PI. Está indicado continuar el proceso diagnóstico, incluyendo métodos invasivos de estudio de la pleura.

The left-sided predominance of asbestos-related pleural disease.

In a series of 406 subjects with a diagnosis of asbestos-related pleural disease (ARPD), the left-right symmetry of radiographically diagnosed ARPD was assessed using the International Labour Organization (ILO) system for classifying radiographic abnormalities and three different statistical models for testing the degree of symmetry. The extent of chest disease was found to be greater on the left than on the right for a number of parameters of pleural disease, including the width and extent of localized pleural thickening, the extent of enface pleural thickening, and the extent of diaphragmatic and chest wall calcification. Current cigarette smoking significantly enhanced the observed asymmetry, with the most pronounced effect being in diaphragmatic calcification. Using a composite scale for each subject's pleural disease, the left side had 1.6 times more localized disease than did the right (p < 0.001). Asymmetry in one parameter was associated with asymmetry in another for a number of pairs. The most significant of these associations was between enface pleural thickening and chest wall calcification (Kendall's tau B = 0.42). The pathophysiologic mechanisms that explain these findings remain elusive and need further investigation.

Pleural lesions and the ILO classification: the need for a revision.

The ILO system for radiographic classification of pneumoconioses is a very important epidemiological tool. Unfortunately, the classification is not precise for pleural lesions, which are especially important for evaluation of asbestos-related diseases. The classification cannot separate extrapleural fat from diffuse thickening of the pleura, nor large plaques from diffuse thickening. In this paper, a short review of the different lesions are given and a revised scheme is suggested. This scheme includes as separate lesions: pleural fat; lesions of the parietal pleura, i.e., pleural plaques; and lesions of the visceral pleura, i.e., diffuse thickening and rounded atelectasis. The extent and width, as in the present ILO scheme, could be abolished.

Asbestos-induced pleural fibrosis and impaired lung function.

To assess the clinical significance of asbestos-induced pleural fibrosis, we evaluated the relationship between radiographic evidence of pleural fibrosis and spirometric values in 1,211 sheet metal workers. Of those with pleural fibrosis (n = 334), 78% had circumscribed plaques and 22% had diffuse pleural thickening involving the costophrenic angle. Factors that were found to be associated with the presence and type of pleural fibrosis included increased age (p less than 0.001), more years in the trade (p less than 0.0001), more years since first exposure to asbestos (p less than 0.0001), more pack-years of cigarette smoking (p less than 0.01), and the presence and degree of interstitial fibrosis (p less than 0.0001). After controlling for these potential confounders (age, years in the trade, latency, pack-years of smoking, and ILO profusion category), linear multivariate regression models demonstrated that both circumscribed plaques (p = 0.007) and diffuse pleural thickening (p = 0.008) were independently associated with decrements in FVC but not with decrements in the FEV1/FVC ratio. Furthermore, our data indicate that the effect of diffuse pleural thickening on decrements in FVC is approximately twice as great as that seen with circumscribed pleural plaques. We conclude that the presence and type of pleural fibrosis among asbestos-exposed workers is independently associated with a pattern of spirometry that is suggestive of an underlying restrictive defect in lung function.

Asbestos-related pleural thickening: effect of threshold criteria on interpretation.

Radiographic evidence of pleural thickening was evaluated in 1,216 shipyard workers (high-risk group) and 214 executives (low-risk group) and classified according to two threshold levels: low (any detectable thickening) and high (thickening less than or equal to 2 mm excluded). Results were markedly different, affecting the low-risk group more than the high-risk group. Changing from the high to the low threshold markedly increased pleural findings and scores as well as inter-reader correlation while reducing both differences between risk groups and detection of additional findings on supplementary oblique views. When data on prevalence and extent of pleural thickening were combined, differences between risk groups were maximized; however, when only prevalence was considered, such differences were minimized, particularly with a low threshold. These findings indicate that different or ambiguous threshold criteria produce divergent results in screening surveys for asbestos-related disease. The authors recommend that explicit minimal pleural threshold criteria be adopted.

Chronic diffuse infiltrative lung disease. Newer approaches.

A new scheme for description of diffuse infiltrative lung diseases using the graphic terminology of the International Labour Office Classification is described. Conventions for grading the type (rounded, or "pqr," and irregular, or "stu"), severity (profusion in 12 steps), localization of opacities, and pleural disease were retained. Modifications included (a) a third group of opacities, called "xyz," corresponding to reticulonodular patterns; and (b) "ground glass" (alveolar) patterns, subdivided into seven types by character and location. In a study of 365 cases proven by open biopsy, when this scheme was used without any knowledge of clinical data, the first two radiologic diagnostic choices corresponded with the principal histologic diagnosis in 50 per cent of cases. This classification provides an understandable and quantifiable system of communication and a tool for teaching, clinical research, and epidemiologic studies.