Idiopathic pulmonary fibrosis is one of these frustrating diseases you repeatedly encounter in the ICU and that mostly leaves you sort of frustrated at the end. Despite all the efforts in research we are still left with very little we can do. This is one reason why also intensivists need to keep themselves updated on this topic.
As knowledge is growing the ATS, ERS, JRS and ALAT (... thoracic and respiratory societies) made the effort to look into the latest evidence by performing systematic reviews and where appropriate meta-analyses. The aim was to update the guidelines published in 2011. These guidelines are also dedicated to Mr. William Cunningham who actively participated in the development of these guidelines, suffered from idiopathic pulmonary fibrosis for many years and who was directly confronted with the issues related with this condition.
The main conclusions can be briefly summarised as follows:
An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline: Treatment of Idiopathic Pulmonary Fibrosis. An Update of the 2011 Clinical Practice Guideline, American Journal of Respiratory and Critical Care Medicine, Vol. 192, No. 2 (2015), pp. e3-e19.
OPEN ACCESS: Executive Summary 2015
An Official ATS/ERS/JRS/ALAT Statement: Idiopathic Pulmonary Fibrosis: Evidence-based Guidelines for Diagnosis and Management, Am J Respir Crit Care Med Vol 183. pp 788–824, 2011 OPEN ACCESS
For further information on acute exacerbations of IPF we recommend this Review Article: