Abstract
Idiopathic pulmonary fibrosis (IPF) is a diffuse, primarily fibroproliferative disorder of lungs characterized by radiologic and histopathologic pattern of usual interstitial pneumonia. IPF is a severe disease which - if untreated - leads inevitably to death.
It is estimated that about 110.000 patients with this disease live in Europe but the count might be even higher due to undiagnosed cases. Median survival of the patients with untreated IPF is 2.5-3 years.
Until very recently, there was no effective drug against this disease. The first drug which directly affects fibroproliferative healing in IPF, pirfenidone, is available in Europe since 2011.
This drug can slow down or stop progression of lung fibrosis and thus slow down decline of lung functions as well. As a result, the prognosis of IPF patients is improved and mortality decreased.
Pirfenidone is reimbursed in the Czech Republic from general health insurance funds since 1. 7. 2014 for the patients with mild or moderate IPF and is available in the Centers for diagnosis and treatment of interstitial lung diseases recognized by Czech Pneumologic and Phtiseologic Society (see www.pneumologie.cz). Every patient with suspected IPF should be sent to some of these centres as early as possible, to be immediately checked and in case of IPF confirmation treated early by specific antifibriotic treatment.
This is the only chance for IPF patients to prolong their survival and to improve the quality of their lives.