PLEUROPARENCHYMAL FIBROELASTOSIS - FIBROSING LUNG DISEASE IN YOUNG ADULTS

Abstract

Purpose of the study. To evaluate the features of the clinical and radiographic picture of pleuroparenchymal fibroelastosis. Materials and methods. From 2006 to 2021 at PSPbGMU im. acad. I.P. Pavlov, 24 patients with a clinical and radiographic picture of pleuroparenchymal fibroelastosis (PPFE) were observed. In all cases, the course of the disease was progressive, the main clinical symptoms were inspiratory or mixed dyspnea during physical exertion up to dyspnea at rest, unproductive cough, weakness, fatigue, weight loss, pneumothorax, as well as radiation and functional signs of pulmonary fibrosis. The average age of the patients was 22.3 ± 10.1 years (w/m - 20/4). All patients underwent traditional X-ray studies (radiography in two projections), high resolution computed tomography (HRCT), a comprehensive pulmonary function tests (PFTs with the determination of DLСО) and echocardiography. In most patients, studies were repeated (on average, CT was performed 4.2). In most cases (0.8), histological verification was performed. Results. Analysis of the results of dynamic examination of patients revealed the following clinical and radiation features of PPFE. 1. Clinical - young people, mostly female, with the onset of the disease in childhood. Then, after a long «light period», partly due to desaggravation associated with the desire of patients to maintain their usual way of life, a progressive course of the disease with severe respiratory failure. 2. Disturbances in the function of pulmonary function tests were in most cases of a mixed nature: a decrease in the vital capacity of the lungs (VC) and total lung capacity (TLC) with an increase or maintenance of the residual volume of the lungs (RLV) at a normal level, it is possible to form an obstructive syndrome mainly at the level of peripheral respiratory pathways (increased OOL and increased bronchial resistance to inhalation and exhalation). All patients had significant or severe disturbances in pulmonary gas exchange (decrease in the diffusion capacity of the lungs). As the disease progressed, there was a negative trend in all indicators of PFTs. 3. Radiographical features: localization of changes in the upper sections, thickening of the apical pleura (apical caps), the presence of large air containing cysts, areas of carnification of the lung tissue (outcomes of organizing pneumonia that occurs at the moments of idiopathic exacerbation, or the addition of a viral infection), the development of pneumothorax and pneumomediastinum. However, during the development of the disease, the manifestation of the classic picture of ordinary interstitial pneumonia (OIP) (“honeycomb lung”) in typical places (lower posterior subleural sections) was noted. In all patients, the manifestations of FBL were accompanied by pronounced manifestations of bronchial obstruction (uneven ventilation of the lung tissue, the presence of «air traps» during a functional CT study «on expiration»), a symptom of three densities («cheese heads»). All patients showed rapid radiation progression of the process (due to young age, often not corresponding to clinical manifestations). Conclusions. Accumulation of experience in clinical and radiological examination of patients with PPFE will make the disease more recognizable, which is important for timely therapy.