Idiopathic pulmonary fibrosis – changes in diagnostics and treatment
Authors:
Vašáková M.
Authors‘ workplace:
Pneumologická klinika 1. LF UK a Thomayerovy nemocnice, Praha
Published in:
Kardiol Rev Int Med 2019, 21(3): 135-141
Idiopatická plicní fibróza (IPF) patří mezi nejobtížněji léčitelné a zároveň nejzávažnější plicní nemoci s prognózou podobnou rakovině plic. Velmi pravděpodobně jsou epidemiologická data v řadě zemí podhodnocena, neboť IPF bývá diagnosticky zaměňována s jinými diagnózami nebo není diagnostikována vůbec. V minulém století, kdy byla poprvé poznána, se jednalo o diagnózu raritní, nyní je nepochybně incidence IPF na vzestupu, na čemž se jistě podílí i zlepšená diagnostika. Původně se jednalo o nemoc neléčitelnou, což se změnilo až v posledních 8 letech s nástupem antifibrotických léků. Díky tomu je dána nemocným šance na lepší přežití, i když zcela vyléčit IPF stále nedovedeme.
Overview
Idiopathic pulmonary fibrosis (IPF) belongs to the most difficult-to-treat and most serious of lung diseases, with prognosis similar to lung cancer. Epidemiologic data are highly probably underestimated in many countries since IPF used to be frequently misdiagnosed for other diagnoses or not diagnosed at all. In the last century, when described for the first time, IPF was an extremely rare diagnosis. Nowadays, the incidence of IPF is undoubtedly rising, which is also supported by improved diagnostic opportunities. Previously, IPF was considered an untreatable disease; however, this has changed in the last eight years as antifibrotic therapy has emerged. Due to this fact, IPF patients have a better chance of survival, although the disease is still uncurable.
Keywords:
idiopathic pulmonary fibrosis – diagnosis
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Paediatric cardiology Internal medicine Cardiac surgery Cardiology Pneumology and ftiseology General practitioner for adults RadiodiagnosticsArticle was published in
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