Idiopatic pulmonary fibrosis – news in multidisciplinary diagnostic and therapeutic approaches

Česká verzia

Authors: Martina Vašáková ¹; Radoslav Matěj ^2,3
Authors place of work: Pneumologická klinika 1. LF UK, Thomayerova nemocnice, Praha ¹; Oddělení patologie a molekulární medicíny, Thomayerova nemocnice, Praha ²; Ústav patologie 1. LF UK a VFN, Praha ³
Published in the journal: Čes.-slov. Patol., 52, 2016, No. 2, p. 85-92
Category: Přehledové články - Interscitiální plicní procesy

Idiopatická plicní fibróza (IPF) je primárně fibrotizujícím plicním procesem a je vzhledem k refrakternosti na dosud známou léčbu a špatné prognóze nejzávažnějším reprezentantem idiopatických intersticiálních pneumonií (IIP). Etiologie a patogeneze onemocnění není zatím plně objasněna, ale předpokládá se, že příčinou fibroproliferace jako odpovědi na neznámý inzult může být nerovnováha reparačních a imunitních dějů u geneticky disponovaného jedince středního a staršího věku.

Summary

Idiopathic pulmonary fibrosis (IPF) is a primary fibrosing pulmonary process. Due to the ineffectiveness of current therapeutic strategies and unfavorable prognosis, IPF is the most serious example of idiopathic interstitial lung diseases (ILD). Etiology and pathogenesis of this disorder have not been fully clarified yet; but it is anticipated, that the fibroproliferation is caused by the imbalance of reparative and immunologic processes in the genetically predisposed patients. Radiologically and histopathologically, IPF is characterized by specific pattern called usual interstitial pneumonia (UIP), however, this pattern is not fully typical in all cases, and, moreover, it could be seen in other ILD´s, e.g. chronic hypersensitivity pneumonitis, asbestosis, autoimmune connective tissue diseases and many others as well. The final diagnosis of IPF is a consensual result of multidisciplinary team composed of pulmologist, pathologist and radiologist. IPF was an incurable disease with prognosis worse than cancer till the year 2011, when antifibrotic drugs decelerating a progression of this disease have been introduced. Earlier and correct diagnosis of IPF is the most important issue for the patients because they could be effectively treated and thus, prolonging their survival as much as possible.

Keywords:
fibrosis – pulmonary – histopathology – diagnosis

Zdroje

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