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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

Pulmonary Fibrosis:Diagnosis 101

Andrew Chan

University of California, Davis

slide2
Consultant and Principal Investigator to Intermune
  • Principal Investigator Actelion and Gilead
slide4

Normal Lung

IPF Lung, UCSF

idiopathic pulmonary fibrosis ipf
Idiopathic Pulmonary Fibrosis (IPF)
  • Age > 50
  • M:F - 2:1
  • Progressive breathlessness
  • Bibasilar crackles, clubbing
  • PERIPHERAL Interstitial pattern
  • Subpleural honeycombing
what symptoms can i expect
What symptoms can I expect?
  • Breathlessness (worse with exercise)
  • Hacking dry cough
  • Fatigue and weakness
  • Appetite and weight loss
  • Enlargement of fingertips (clubbing)
am i just unfortunate
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
what causes pulmonary fibrosis 200 causes
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

slide10
A detailed history is very important including previous occupations and exposures.
  • Emphasis on an Early and Accurate diagnosis
silicosis
Silicosis

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

slide12

NORMAL

SILICOSIS

http://brighamrad.harvard.edu/fireCases/images/42/cxr521.gif

www.learningradiology.com

sarcoidosis
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

scleroderma
Scleroderma

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

serologic tests can help exclude other conditions
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.

6 minute walk test
6-Minute Walk Test

www.pspsj.com

bronchoscopy
Bronchoscopy

www.pcca.net/images/BronchoscopyNose.jpg

one example of pf ipf
One Example of PF-IPF
  • IPF is the commonest idiopathic ILD
early hrct findings in ipf
Early HRCT Findings in IPF

Courtesy of David A. Lynch, MD.

slide23

UIP: Traction Bronchiectasis

Courtesy of W. Richard Webb, MD.

international concensus statement on ipf histology
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.

video assisted thoracoscopic surgery vats
Video-Assisted Thoracoscopic Surgery (VATS)

www.mayoclinic.org/video-assisted-thoracic-surgery/

approach to the diagnosis of ipf
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

slide27
“It is far better to cure at the beginning than at the end” Aulus Persius Flaccus, Roman poet, A.D. 34–A.D. 62