Klin Farmakol Farm. 2016;30(1):30-34 | DOI: 10.36290/far.2016.006

Current pharmacotherapy of idiopathic pulmonary fibrosis

Martina Vašáková

Pneumologická klinika 1. LF UK, Thomayerova nemocnice Praha

Idiopathic pulmonary fibrosis (IPF) belongs to pulmonary diseases with a grim prognosis and challenging treatment. It is characterized

by progressive breathlessness and physical signs of clubbed fingers and crepitus found on auscultation at lung bases.

Radiologic and histopathologic findings of IPF correspond to those of usual interstitial pneumonia. The mean survival of IPF patients

is 2.5 years. Until recently, no efficient therapy of IPF existed. The year 2011 became a milestone in IPF treatment when the

first antifibrotic drug, pirfenidone, was registered in most European countries. In 2015, the second antifibrotic drug, nintedanib,

came to Europe. Coincident with the development of treatment options in IPF is striving for early diagnosis of IPF in order to

provide more and more patients with efficient treatment. However, it must be realized that any antifibrotic treatment only slows

down the disease progression, but cannot stop it. It means that the earlier we establish the diagnosis of IPF and start antifibrotic

treatment, the longer survival can be offered to IPF patients.

Keywords: idiopathic pulmonary fibrosis, early diagnosis, antifibrotic treatment, pirfenidone, nintedanib

Published: May 30, 2016  Show citation