Welcome to Louisville! Where is Louisville? Founded in 1778 by George Rogers Clark Healthcare, Humana, YUM, Ford, UPS Abraham Lincoln William Clark Zachary Taylor (12th) Victor Mature Bob Edwards Pee Wee Reese Abraham Flexner Old Med School Bucks for Brains Late 1990s HCOC, 2008 JBCC Renovation 2007 Clin Trans Res Build, 2009 John Walker Moore, M.D. (1884-1952) Nucleus, 2014 72 y/o male with cough A B C • Interstitial lung disease • Phys exam: Lung crackles and clubbing • Imaging: Chest CT with peripheral and basilar infiltrates, honeycombing, traction bronchiectasis • PFTs: Restriction • Pathology - UIP Idiopathic Pulmonary Fibrosis Idiopathic Pulmonary Fibrosis has become the best studied interstitial lung disease We know in who to suspect it. Determine cause / etiology We know how to diagnose it. Develop animal models We know how to distinguish it from other Identify effective treatments disorders characterized by lung fibrosis. Identify biomarkers to facilitate We know its prognosis. diagnosis or for testing new treatments We know how to treat its complications. Translate what we have learned to the study of related conditions Continue to increase awareness 70 y/o male with cough • Interstitial lung disease • Phys exam: Lung crackles • Imaging: CT with bilateral infiltrates • PFTs: uncooperative Idiopathic Pulmonary Fibrosis • Pathology: Architectural distortion and areas of fibrosis Idiopathic Pulmonary Fibrosis has become the best studied interstitial lung disease We don’t know when to suspect it. We don’t know how to diagnose it. We don’t have great ways of distinguishing it from other entities. We don’t know its prognosis. We may not be able to treat complications. Determine cause / etiology Develop animal models Identify effective treatments Identify biomarkers to facilitate diagnosis or for testing new treatments Translate what we have learned to the study of related conditions Continue to increase awareness Can we bring these two areas together with the intention of accelerating discovery? Historical Background Oct 2007 Meeting in Lafayette, IN (WFA; Wayne Kompare, President) May 2008-09 ATS Working Group established (RCMB and Clin Prob) May 2009 ATS funds project and working group meets in San Diego, CA May 2010 Meeting of working group in New Orleans, LA Dec 2013 ATS publication Apr 2014 FAS meeting Oct 2014 Document ready for submission 2015 Publication Comparative Pathobiology of Fibrosing Lung Disorders in Humans and Domestic Animals Annals ATS 2013 Recommendation #1 - Detailed descriptive studies should be conducted in affected domestic animals to define the clinical, imaging, and pathological presentation of pulmonary fibrosis in these species. Recommendation #2 - Genetic studies and other pathogenesisbased investigations should be conducted in naturally occurring spontaneous models of pulmonary fibrosis to investigate the potential translation to IPF in humans. Comparative Pathobiology of Fibrosing Lung Disorders in Humans and Domestic Animals Annals ATS 2013 Recommendations #3 and #4 - Studies should be conducted to define the anatomical and cellular differences in the lungs of different species for the adequate interpretation of discordant findings. Suitable reagents (and models) needed to adequately test hypothesis in different species of animals should be generated. Recommendation #5 - A consortium of interested centers and a central repository of clinical information and biologic specimens from naturallyoccurring spontaneous models of lung fibrosis in domestic animals should be established to enable further research that may benefit both physicians and veterinarians in their efforts to adequately manage lung fibrosis in their patient populations.