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Clinical trials in idiopathic pulmonary fibrosis: update and perspectives

Eva Baroke, Shyam Maharaj, Martin Kolb

Idiopathic pulmonary fibrosis (IPF) is an increasingly prevalent and devastating disease with high mortality and poor treatment options. Major advances in understanding its underlying pathogenesis have changed the traditional concept of IPF being a chronic inflammatory condition to it being a disordered wound healing and repair process, and have enabled the development of novel therapeutic drug strategies. Over the last decade, a number of well-designed clinical trials have been conducted without any tremendous breakthrough to date. Nevertheless, the activities have dramatically improved clinical and basic understanding of IPF and resulted in approval of the first IPF-specific therapy. This review summarizes the most important clinical trials in IPF and outlines both novel drug targets and challenges for future trials.