Idiopathic Pulmonary Fibrosis: Epidemiology, Diagnosis andOutcomes

Abstract

The incidence and prevalence of idiopathic pulmonary fibrosis (IPF) is increasing worldwide. This, combined with its poor prognosis and unpredictable natural history, has amplified the importance of an accurate diagnosis and monitoring. A diagnosis of exclusion, IPF requires a comprehensive clinical evaluation. This results in a clinical context that provides the backdrop for interpretation of the chest imaging and histopathology. A confident or probable usual interstitial pneumonia chest imaging pattern on high-resolution computerized tomography may be diagnostic in the correct clinical context. Outcomes for IPF are unpredictable, ranging from rapid progression with death within months to prolonged stability. Disease activity is monitored by clinical and physiology measures with a declining forced vital capacity, a recognized measure of progression. The available treatments, pirfenidone and nintedanib, are effective at reducing the expected decline in forced vital capacity.