Welcome to Louisville!

2. Welcome to Louisville!

3. Where is Louisville? Founded in 1778 by George Rogers Clark

4. Healthcare, Humana, YUM, Ford, UPS

5. Abraham Lincoln William Clark Zachary Taylor (12th) Victor Mature Bob Edwards Pee Wee Reese Abraham Flexner

6. Old Med School Bucks for Brains Late 1990s HCOC, 2008 JBCC Renovation 2007 Nucleus, 2014 Clin Trans Res Build, 2009 John Walker Moore, M.D. (1884-1952)

7. 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

8. Idiopathic Pulmonary Fibrosis has become the best studied interstitial lung disease 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 We know in who to suspect it. We know how to diagnose it. We know how to distinguish it from other disorders characterized by lung fibrosis. We know its prognosis. We know how to treat its complications.

9. 70 y/o male with cough • Interstitial lung disease • Phys exam: Lung crackles • Imaging: CT with bilateral infiltrates • PFTs: uncooperative • Pathology: Architectural distortion and areas of fibrosis Idiopathic Pulmonary Fibrosis

10. 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?

11. Historical Background • Oct 2007 Meeting in Lafayette, IN (WFA; Wayne Kompare, President) • May 2008-09 ATS Working Group established (RCMB and ClinProb) • 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

12. 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 pathogenesis-based investigations should be conducted in naturally occurring spontaneous models of pulmonary fibrosis to investigate the potential translation to IPF in humans.

13. 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 naturally-occurring 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.