Idiopathic pulmonary fibrosis (IPF) involves chronic fibrosing interstitial pneumonia of unknown cause and shows a progressive worsening in lung function and dyspnea. The prognosis is similar to many cancers. Therapies: Nintedanib and Pirfenidone, have been recently approved. Diagnosis IPF is highly heterogeneous with varying rates of clinical progression. Decline in lung function and response to therapies.
Lung tissue shows a characteristic histopathologic pattern: Usual interstitial pneumonia (UIP), with areas of normal parenchyma interspersed with areas of paraseptal and subpleural fibrosis, and architectural distortion within these areas. Presence of fibroblastic foci, honeycomb cysts, and immune cell involvement is also possible. Clinical trials IPF has a history of landmark negative clinical trials. Heterogeneity is thought to be a key reason in the negative outcomes. Novel…
