Idiopathic pulmonary fibrosis: an update

摘要

Idiopathic pulmonary fibrosis (IPF) is a chronic lung condition of uncertain aetiology that should be considered in the differential diagnosis of patients who experience breathlessness, cough and reduced exercise tolerance. IPF is characterized histologically by the presence of usual interstitial pneumonia, and often has typical radiological appearances. Long-term successful management options are limited and frequently unsuccessful; as the disease progresses, palliation of symptoms becomes the mainstay of treatment. In a minority of patients, lung transplantation provides the only hope of long-term survival. The median survival of patients with IPF is approximately 3 years, which in turn emphasizes the need for further investigation into its pathogenesis and potential disease-modifying pharmacological therapies.