Description
The medium survival of idiopathic pulmonary fibrosis (IPF) patients is 2.5 to 3.5 years after diagnosis. In the U.S. alone, approximately 40,000 people die of IPF annually. The pathogenesis of IPF is only partially understood and the cause(s) of IPF are not yet known. Although a number of IPF clinical trials are currently ongoing, there are still no proven medical therapies for this lethal disease. In fact, the results from the latest phase III clinical trial sponsored by NHLBI (the PANTHER-IPF study) have shown that a commonly prescribed regimen consisting of prednisone, azathioprine and n-acetylcysteine is harmful in IPF patients. Thus, lung transplantation remains the only therapeutic option available for a small fraction of end-stage IPF patients.
To facilitate development of novel therapies for IPF based on a better understanding of its pathogenesis, NHLBI convened a workshop on November 27-28, 2012 to draft a strategic plan for future IPF research. The workshop brought together basic, translational and clinical pulmonary fibrosis investigators along with pharmaceutical, FDA and patient advocacy group representatives to discuss 1) knowledge gaps in IPF pathogenesis, 2) unmet needs in IPF research, 3) obstacles to translating basic discoveries into clinical tools/therapies, 4) potential collaborations and coordination of research efforts.