Idiopathic pulmonary fibrosis

D. Keating, B. McCullagh, J. Egan

Research output: Chapter in Book/Report/Conference proceedingChapter (Book)Researchpeer-review


Idiopathic pulmonary fibrosis (IPF) is an increasingly common condition which poses many challenges. High resolution CT scanning offers a non-invasive technique which can secure an accurate diagnosis in the majority of patients. Lung function estimates of gas transfer allow the definition of advanced (DLco < 39% predicted) and limited disease (DLco > 40% predicted).Developments in the area of medical therapy have been slow. Based on current thinking regarding the pathophysiology of IPF, steroids are increasingly seen as having only a limited role in its treatment. Emerging studies are highlighting the importance of alternative forms of therapy including N-acetylcysteine, pirfenidone and bosentan, for example. Lung transplantation is a legitimate surgical treatment for IPF. However, in light of the high death rate on the waiting list, it is recommended that selected patients are referred early to the transplant programme.

Original languageEnglish
Title of host publicationDiffuse Parenchymal Lung Disease
EditorsUlrich Costabel, Jim Egan, Roland Bois
Number of pages12
Publication statusPublished - 2007
Externally publishedYes

Publication series

NameProgress in Respiratory Research
ISSN (Print)1422-2140
ISSN (Electronic)1662-3932

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