Idiopathic pulmonary fibrosis and progressive fibrotic interstitial lung disease

Abstract

Interstitial lung diseases (ILDs) represent a spectrum of disorders affecting the lung parenchyma. The most common of the idiopathic interstitial pneumonias is idiopathic pulmonary fibrosis (IPF), characterized by progressive lung fibrosis, lung function decline, symptom burden and early mortality. The precise aetiology of IPF remains unknown and there are currently no curative treatment options; management strategies focus on symptom control and slowing disease progression with antifibrotic medication. Despite the diversity of pathological and clinical features seen in non-IPF interstitial lung diseases, a subset of patients with these conditions manifest a progressive fibrotic course that is similar to those with IPF. This clinical phenotype has been described as progressive fibrotic ILD (PF-ILD) and is defined by clinical, physiological and radiological progression. This article provides an overview of IPF and PF-ILD including the diagnostic process, typical disease course and management approach.