Transcript Louisville - Westie Foundation of America

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.