Abstract
Idiopathic pulmonary fibrosis (IPF) is a primarily fibrosing lung process that represents the most severe form of idiopathic interstitial pneumonias due to its refractoriness to therapies known so far and its poor prognosis. Neither the aetiology nor pathogenesis of the disease has been fully clarified so far, but the anticipated cause of fibroproliferation as a response to unknown insult might be imbalance between reparation and immune processes in genetically predisposed middle age and elderly individuals.
Until 2011 no effective therapy of the disease has been available in the Czech Republic (CR), and patients stood no chance of extended survival unless they could become candidates for lung transplant. Since 2011 pirfenidone has been available in the CR, a truly breakthrough drug for the treatment of IPF.
It is the first drug to have been shown effective in terms of slowing down declining pulmonary functions in patients with IPF, effectively shifting the incurable disease among curable conditions. A second drug available for patients with IPF since 2014 as early therapeutic approach and at least comparable with pirfenidone in its efficacy is nintedanib.
It is important to realize, however, that both these agents only slow down the process of deterioration of pulmonary functions, meaning most benefit is to be expected in patients with early diagnosis and timely initiation of treatment. In the CR, therapy of IPF is centralized in Centres for Diagnosis and Treatment of Interstitial Lung Processes where each patient should be sent as soon as possible after he or she is suspected to suffer from IPF.
Data on patients and treatment progress are entered anonymously in IPF Registry. This makes it possible to follow-up the efficacy of therapy of IPF in our country under real-life conditions.