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Pulmonary Fibrosis: Diagnosis 101

Pulmonary Fibrosis: Diagnosis 101. Andrew Chan University of California, Davis. Consultant and Principal Investigator to Intermune Principal Investigator Actelion and Gilead. What is Pulmonary Fibrosis (PF)?. Scarring of the lungs. Normal Lung. IPF Lung, UCSF.

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Pulmonary Fibrosis: Diagnosis 101

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  1. Pulmonary Fibrosis:Diagnosis 101 Andrew Chan University of California, Davis

  2. Consultant and Principal Investigator to Intermune • Principal Investigator Actelion and Gilead

  3. What is Pulmonary Fibrosis (PF)? • Scarring of the lungs

  4. Normal Lung IPF Lung, UCSF

  5. Idiopathic Pulmonary Fibrosis (IPF) • Age > 50 • M:F - 2:1 • Progressive breathlessness • Bibasilar crackles, clubbing • PERIPHERAL Interstitial pattern • Subpleural honeycombing

  6. What symptoms can I expect? • Breathlessness (worse with exercise) • Hacking dry cough • Fatigue and weakness • Appetite and weight loss • Enlargement of fingertips (clubbing)

  7. Am I just unfortunate? • Over 5 million people worldwide have fibrosis • Over 300,000 in the US (likely more) • 100,000 new cases every year • 40,000 die each year (same as breast cancer) perhaps more (51/1,000 000 people) • UK- one in 3,000 has PF. • 3,000 die annually from PF

  8. What causes Pulmonary Fibrosis?(200 causes!) 1) Occupational & Environmental – Silicosis, Asbestosis, Hypersensitivity Pneumonitis 2) Drug Induced – Amiodarone, Nitrofurantoin, Methotrexate, Cocaine 3) Connective Tissue Diseases – Lupus, RA, Scleroderma 4) Primary Diseases – Sarcoidosis, LAM 5) Idiopathic (25%) – IPF, NSIP 6) Genetics soweirdo.com

  9. How do we narrow this list to the actual cause in me?

  10. A detailed history is very important including previous occupations and exposures. • Emphasis on an Early and Accurate diagnosis

  11. Silicosis www.cdc.gov/Spanish/niosh/images/silifig0.gif

  12. NORMAL SILICOSIS http://brighamrad.harvard.edu/fireCases/images/42/cxr521.gif www.learningradiology.com

  13. www.cowart.info/.../Parrotman-782740.JPG

  14. Physical Examination can be diagnostic.

  15. Sarcoidosis myweb.lsbu.ac.uk/dirt/museum/margaret/68--223-2001241.jpg myweb.lsbu.ac.uk/dirt/museum/margaret/68--223-2001241.jpg www.dwp.gov.uk/medical/med_conditions/images/sarcoidosis3.jpg

  16. Scleroderma newsimg.bbc.co.uk/media/images/39397000/jpg/

  17. Serologic Tests Can Help ExcludeOther Conditions ESR, ANCA ANA CCP (for RA) CK Aldolase Anti-myositis panel with Jo-1 antibody ENA panel • Scl-70 • Ro (SSA) • La (SSB) • Smith • RNP Connective tissue diseases Hypersensitivity pneumonitis Hypersensitivity panel (if exposure history) ATS/ERS. Am J Respir Crit Care Med. 2000;161:646-664.

  18. Focus on the TLC and DLCO, both low

  19. 6-Minute Walk Test www.pspsj.com

  20. Bronchoscopy www.pcca.net/images/BronchoscopyNose.jpg

  21. One Example of PF-IPF • IPF is the commonest idiopathic ILD

  22. Early HRCT Findings in IPF Courtesy of David A. Lynch, MD.

  23. UIP: Traction Bronchiectasis Courtesy of W. Richard Webb, MD.

  24. International Concensus Statement on IPF: Histology • Surgical lung biopsy (VATS) recommended in patients with suspected IPF, especially those with atypical clinical or radiographic features ATS/ERS.Am J Respir Crit Care Med. 2000;161:646.

  25. Video-Assisted Thoracoscopic Surgery (VATS) www.mayoclinic.org/video-assisted-thoracic-surgery/

  26. Approach to the Diagnosis of IPF • Clinical • History • Physical • Laboratory • PFTs • Radiology • Chest X-ray • HRCT • Pathology • Surgical lung biopsy Primary care physicians Pulmonologists Radiologists Pathologists Multidisciplinary

  27. “It is far better to cure at the beginning than at the end” Aulus Persius Flaccus, Roman poet, A.D. 34–A.D. 62

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