The European IPF Network: Towards better care for a dreadful disease

  • Guenther A
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Idiopathic pulmonary fibrosis (IPF) is one of the most aggressive forms of diffuse parenchymal lung disease (DPLD) and probably affects up to 340,000 people in Europe and North America 1. Despite extensive research over the past 25 yrs, considerable investment in controlled clinical trials, and significant progress in defining appropriate outcome measures and surrogates of disease progression, IPF remains a progressive and invariably fatal disease with a mean survival of ∼3 yrs from diagnosis. With the exception of pirfenidone 2, which is now on the way of being approved in Europe, and N-acetylcysteine 3, which has shown some signs of therapeutic efficacy in IPF, no other investigational agents, including bosentan 4, imatinib 5 and interferon-γ 6, have proven to be beneficial in phase III studies performed over the last decade. IPF, therefore, remains a dismal disease for which new effective therapeutic approaches are urgently needed. Despite this, we would argue that there is much cause to be optimistic in terms of the current climate for scientists and clinicians striving to develop novel therapeutic strategies for this devastating disease. The last 5 yrs or so have seen major advances, including the definition of novel paradigms of disease pathogenesis and the discovery of novel biomarkers. Pharmaceutical interest and investment from both academia and industry are at unprecedented levels. We must, however, be careful to heed the lessons we have learned from our past experience. WHY HAVE SO MANY TRIALS IN IPF FAILED AND WHY HAVE THERE BEEN NO MAJOR BREAKTHROUGHS IN THE TREATMENT OF IPF? There are many possible answers to these important questions. First, we need to deconstruct the way IPF has been approached in terms of controlled clinical trials. Most of the trials conducted to date were based on an “opportunistic approach”, using drugs or investigational compounds developed for other disease indications rather than agents which emerged from a systematic programme for the development of novel … EXCLUSION: Focused on medical content

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  • A. Guenther

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