ALVEOLAR PULMONARY PROTEINOSIS (LITERATURE REVIEW, CLINICAL-RADIOLOGIC OBSERVANCE, ASSESSMENT OF THE COURSE DYNAMICS)

In standard classifications of interstitial pulmonary diseases alveolar pulmonary proteinosis (APP) by etiologic signs is referred to the group of idiopathic ones, where besides APP there is sarcoidosis, idiopathic pneumofibrosis, Hand-Schuller-Christian disease, idiopathic interstitial alveolitis (acute interstitial pneumonia, Hamman-Rich syndrome) and others. APP is a rare but potentially curable disease of unknown etiology, characterized by the disorder of surfactant metabolism, which leads to accumulation of protein in alveoli (superficially active substances) and its components, including phospholipids. The main feature of APP (and this is definitely taken into account in diagnostics) is its poor clinical picture which does not correspond to large pulmonary damages registered in roentgenograms and at multispiral computer-aided tomography (MSCT) with post processing image processing. This article gives a review of recently published domestic and foreign literature. During 5 years we examined, treated and observed in dynamics 6 patients with APP. This report presents one research whose feature is the wrong clinical diagnosing of the disease at all preliminary stages of examination of the patient with APP. Because of clinical mistakes open biopsy was done in a wrong way, without special painting of biopsy material upon the presence of protein components. As a result of system mistakes several courses of improper therapy were done, which led to complications of the disease in the form of recurrent pneumonia. Final clinical-radiologic examination allowed to diagnose autoimmune form of APP, and the repeat open biopsy with the painting of biopsy material with Schiff's reagent (PAS-reaction) gave us a chance to make a final diagnose. At dynamic monitoring with the help of MSCT in 6 months this patient had almost complete spontaneous APP remission.

альвеолярный легочный протеиноз, лучевая диагностика, мультиспиральная компьютерная томография, биопсия, PAS-реакция, pulmonary alveolar proteinosis, roentgen diagnostics, computer-aided tomography, biopsy, PAS reaction

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