Idiopathic Pulmonary Fibrosis

Excerpt

Idiopathic pulmonary fibrosis (IPF) is a lung disorder where there is scarring of the lungs from an unknown cause. It is usually a progressive disease with a poor long-term prognosis. The classic features of the disorder include progressive dyspnea and a nonproductive cough. Pulmonary function tests usually reveal restrictive impairment and diminished carbon monoxide diffusing capacity. The diagnosis can be made without biopsy if there are compatible imaging tests, appropriate clinical history, and exclusion of other conditions. The classic pattern of imaging on computed tomography (CT) scan will show a peripheral distribution of bilateral fibrosis, more pronounced at the bases. If there is diagnostic uncertainty, IPF can also be diagnosed by lung biopsy. The treatment includes supportive measures, oxygen supplementation when needed, anti-fibrotic drugs, and lung transplants for severe disease.