Idiopathic Pulmonary Fibrosis: An Overview

Idiopathic pulmonary fibrosis (IPF) is the prototypical fibrosing interstitial lung disease, characterised by its unrelenting progressive course and poor prognosis. The incidence of IPF is rising and is becoming a major public health concern. Debilitating dyspnoea and respiratory failure results. Death occurs on average 3-5 years from the time of diagnosis. Clearer understanding of the pathobiology of the condition continues to advance and although we may now better understand disease mechanisms, our therapeutic approach remains limited. In the UK, only two treatments are licensed for IPF, and both can only slow the process down. An ideal silver bullet would halt and ideally reverse established fibrosis. New therapies are showing promise; however, lung transplant remains the only treatment that can substantially increase both duration and quality of life. Here we will provide a comprehensive overview of IPF to summarise definitions, epidemiology, mechanisms, diagnostics, and management.