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Abstract
The idiopathic interstitial pneumonias can be grouped, for treatment purposes, into primary inflammatory disorders, fibrotic nonspecific interstitial pneumonia (in which inflammation is thought to precede and progress to fibrosis) as well as the most common of the idiopathic interstitial pneumonia subgroups, idiopathic pulmonary fibrosis. Over the past decade, there have been several paradigm shifts in the understanding of idiopathic interstitial pneumonias and their treatment. In particular, we highlight changes in the use of prognostic markers, clinical trial end points and the understanding of pathogenesis of idiopathic pulmonary fibrosis. We outline a practical approach to the treatment of these three patient groups.
Financial & competing interests disclosure
AU Wells has received, from Actelion, a research grant and fees for participating as a speaker in international symposia. He has also participated in steering committees, with input into study design for Actelion, Encysive, Centocorp and Genzyme. The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.
No writing assistance was utilized in the production of this manuscript.