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Treatment of idiopathic interstitial pneumonias

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.