Interní Med. 2007; 9(5): 233-236

Idiopathic pulmonary fibrosis - current concepts in diagnosis and treatment

MUDr. Martina Vašáková

Anesteziologicko resuscitační klinika 1. LF UK a FTNsP Praha, Pneumologická klinika 1. LF UK a FTNsP Praha

Idiopathic pulmonary fibrosis (IPF) is a serious disease with a grim prognosis with median survival 3–5 years despite any known treatment. Etiology of the disease has not been satisfactory ellucidated to date. The imbalance of immune response with prevalence of Th2 type is supposed to play crucial role in uncontrolled fibroproduction as a response to unknown stilumulus. IPF is presenting with progressive exertion dypnea. The typical physical sign is finger – clubbing and bilateral basal crackles on auscultation. The dominant radiologic finding are widening of interlobular septs and honey-combing, lunfg function tests show restrictive ventilatory pattern with reduced diffusing capacity and compliance. The counts of polymorphonuclear cells are increased and eosinophils can be present in bronchoalveolar lavage fluid. The histologic finding from lung biopsy shows the pattern of usual interstitial pneumonitis. No effective treatment of IPF extending life-expectancy is known to date. The standard treatment regimens include corticosteroids, immunosuppressive drugs (azathioprin, cyclophosphamide) and acetylcystein as potent antioxidant agent. Frequently, lung transplantation seems to be the only effective treatment for suitable IPF patients.

Keywords: idiopathic pulmonary fibrosis, diagnosis, treatment

Published: June 21, 2007  Show citation