Idiopathic pulmonary fibrosis: in need of focused and systematic management.

Abstract

Idiopathic pulmonary fibrosis (IPF) is an increasingly recognised, serious lung disease. A recent International Consensus Statement has redefined the term "idiopathic pulmonary fibrosis", restricting its use to the entity previously described as "usual interstitial pneumonia" and reclassifying some of the more benign inerstitial lung diseases formerly included under IPF. There is insufficient quality evidence for the effectiveness of current medical therapies for IPF. Lung transplantation provides a potential surgical therapeutic option for selected individuals with IPF, but referral for transplant needs to be made as early as possible. Multidisciplinary clinics specialising in interstitial lung disease have a potential role in determining which patients may benefit from novel and existing medical therapies and which patients should be referred for lung transplantation.