Idiopathic pulmonary fibrosis (IPF) is a devastating lung disease of unknown 
cause. The clinical course is unpredictable, but the disease is usually 
progressive with a median survival of 2-5 years as a result of advanced 
respiratory failure. The current hypothesis of the disease mechanism is 
recurrent injury to the respiratory epithelium which leads to an uncontrolled 
wound healing process resulting in fibrosis rather than repair. Despite better 
understanding of the pathogenesis, there is no effective therapy for the 
disease. In this review, we present the positive results of recently published 
clinical trials regarding therapy for IPF, with emphasis on pirfenidone and 
nintedanib.