Idiopathic pulmonary fibrosis (IPF) is the most common subtype of usual interstitial pneumonias (UIP); it usually leads to progressive pulmonary fibrosis. Aetiology is unknown and the prognosis is poor in the majority of cases; however, the course of the disease shows significant variation. Signs and symptoms are often initially nonspecific, and delays in diagnosis are therefore common. Diagnosis is confirmed in specialist health care. No curative drug therapy is known as yet. Pirfenidone is currently the only medication indicated in pulmonary fibrosis, and it may slow disease progress in some patients. A lung transplant is a feasible treatment option in some cases.