Idiopathic pulmonary fibrosis (IPF) is an increasingly recognised, serious
lung disease.
A recent International Consensus Statement has redefined the term "idiopath
ic pulmonary fibrosis", restricting its use to the entity previously descri
bed as "usual interstitial pneumonia" and reclassifying some of the more be
nign inerstitial lung diseases formerly included under IPF.
There is insufficient quality evidence for the effectiveness of current med
ical therapies for IPF.
Lung transplantation provides a potential surgical therapeutic option for s
elected 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 exi
sting medical therapies and which patients should be referred for lung tran
splantation.