Idiopathic pulmonary fibrosis. Can we always diagnose and treat it right?

Czech version

Authors: Martina Vašáková
Authors‘ workplace: Pneumologická klinika 1. LF UK a Thomayerovy nemocnice Praha
Published in: Vnitř Lék 2017; 63(11): 796-801
Category: Reviews

Idiopatická plicní fibróza (IPF) je závažné plicní onemocnění charakterizované progresivní fibrotizací plicní tkáně na podkladě patologického vzoru hojení alveolárních lézí. Onemocnění vzniká u jedinců středního a staršího věku, kteří nesou genetickou dispozici k fibroproliferaci. Klinický obraz není jednotný. Pacienti mohou mít rychle progredující onemocnění, případně mohou mít závažné akutní exacerbace, ale mohou mít i relativně benigní průběh nemoci. Problémy v diagnostice činí netypické radiologické obrazy nemoci a nemožnost provést plicní biopsii, případně nediagnostická plicní biopsie. Kauzální léčba této smrtící nemoci je dostupná od roku 2011, nicméně nevede k vyléčení, ale pouze ke zpomalení poklesu plicních funkcí a prodloužení přežití.

Overview

Idiopathic pulmonary fibrosis (IPF) is a severe pulmonary disease characterized by fibrotisation of lung tissue based on pathological healing of alveolar lesions. The disease is initiated in individuals of middle and older age who have a genetical disposition to fibroproliferation. Clinical presentation is not uniform. The patients can have a rapidly progressive disease, eventually severe acute exacerbations, however some of them can have relatively benign course of the disease. Problems in diagnosis are usually caused by atypical radiologic findings and no or nondiagnostic lung biopsy. Causal treatment of this life-threatening disease is available since 2011, nevertheless this treatment is not curative but only slows down declination of lung function and prolongation of survival. Thus it is important to identify the patients with IPF and start treatment early. Section for interstitial lung diseases of the Czech pneumologic and Phtiseologic Society founded the registry of idiopathic pulmonary fibrosis patients (EMPIRE), which has been then adopted by other European and Asian countries and become the biggest registry of this disease in the world. The registry can offer an answer on many questions about this disease in real world and thus is helpful for further knowledge of idiopathic pulmonary fibrosis included the atypical manifestations.

Key words:
atypical clinical and radiologic manifestation –⁠ idiopathic pulmonary fibrosis –⁠ registry EMPIRE

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