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IPF Diagnosis: A Practical, Multidisciplinary Approach

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Title: IPF Diagnosis: A Practical, Multidisciplinary Approach


1
IPF Diagnosis A Practical, Multidisciplinary
Approach
Learning Objective Adopt into clinical practice
a multidisciplinary approach to diagnosing
patients with IPF
2
Current Definition of IPF
  • Distinct chronic fibrosing interstitial pneumonia
  • Unknown cause
  • Limited to the lungs
  • Has typical HRCT findings
  • Associated with a histologic pattern of UIP

ATS/ERS Consensus Statement. Am J Respir Crit
Care Med. 2002165277-304.
3
US Demographics of IPF
Updated!
  • Incidence gt 30,000 patients/year
  • Prevalence gt 80,000 current patients
  • Age of onset most 4070 years
  • Two-thirds gt 60 years old at presentation
  • Males gt females

ATS/ERS. Am J Respir Crit Care Med.
2000161646-664. Raghu G, et al. Am J Respir
Crit Care Med. 2006174810-816.
4
Risk Factors for IPF
Updated!
  • Risk factors
  • Familial
  • Smoking
  • Associated factors
  • Environment (eg, wood or metal dust)
  • Gastroesophageal reflux disease (GERD)
  • Infectious agents

ATS/ERS. Am J Respir Crit Care Med.
2000161646-664. Raghu G, et al. Am J Respir
Crit Care Med. 2006174810-816.
5
Pulmonary Function Tests
  • Normal PFTs or resting ABG do not exclude IPF
  • Restriction (not always seen)
  • Reduced FVC and TLC
  • Normal or increased FEV1/FVC ratio
  • Impaired gas exchange
  • Decreased DLCO, PaO2
  • Desaturation on exercise oximetry
  • Increased P(A-a)O2 gradient

ATS/ERS. Am J Respir Crit Care Med.
2000161646-664.
6
IIP Classification
HC, honeycombing GGO, ground glass opacity FF,
fibrotic foci M?, macrophage
Adapted from Thannickal VJ, et al. Annu Rev Med.
200455395-417. Adapted from Strollo DC. Am J
Respir Cell Mol Biol. 200329(3 Suppl)S10-S18.
7
Diagnosing Chronic Exertional Dyspnea
Updated!
Differential Diagnosis
Neuromuscular
Pulmonary
Cardiac
Vascular
Others
NM disease, Malnutrition, Diaphragm dysfunction
Pulmonary Hypertension AVM
Anemia, Anxiety, Obesity, Deconditioning,
Hyperthyroidism
Asthma, COPD, ILD
Cardiomyopathy, R-to-L shunt
Basilar Velcro crackles?
yes
no
ILD
Asthma, COPD
Typical HRCT scan
Confident diagnosis of IPF
Image courtesy of Steven A. Sahn, MD.
8
Biomarkers for IPF
Updated!
Research findings, pending clinical validation
  • Matrix Metallo-Proteases (MMP1/MMP7)1
  • Surfactant proteins A D2
  • TGFb-13
  • KL-64
  • Collagen turnover products (PIIINP, ICTP,
    PYD/DYD)5
  • Rosas IO, et al. PLoS Med. 20085e93.
  • Nakamura M, et al. Nihon Kokyuki Gakkai Zasshi.
    2007 Jun45(6)455-459.
  • Greene KE, et al. Eur Respir J. 200219439-446.
  • Allam JS, et al. ATS 2008 poster 919.
  • Yokoyama A, et al. Am J Respir Crit Care
    Med.19981581680-1684.
  • Schaberg T, et al. Eur Respir J.199471221-1226.
    Hiwatari N, et al. Tohoku J Exp Med.
    1997181(2)285-95. Froese AR, et al. ATS 2008
    poster 907.

9
Serologic Tests Can Help ExcludeOther Conditions
Updated!
  • ESR
  • 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.
2000161646-664.
10
IPF Radiographic Findings
IPF cannot be ruled out by a normal chest
radiograph
16 of patients with ILD have normal chest
radiographs
Orens JB, et al. Chest. 1995108109-115.
11
Chest Radiograph in IPF
Basal and peripheral reticulation
Reduced lung volume
Images courtesy of W. Richard Webb, MD.
12
HRCT Diagnosis of IPF
Prone scans are best for showing subtle
abnormalities
ATS/ERS. Am J Respir Crit Care Med.
2000161646-664.
13
Early/Low Burden IPF HRCT
Reticular opacities with a subpleural and basal
predominance
No honeycombing or traction bronchiectasis
Image courtesy of W. Richard Webb, MD.
14
Classic IPF HRCT
Basal and subpleural predominance
Reticular opacities
Traction bronchiectasis
Honeycombing
Image courtesy of W. Richard Webb, MD.
15
Advanced IPF HRCT
Extensivehoneycombing
Traction bronchiectasis
Reticular opacities
Basal and subpleural predominance
Image courtesy of W. Richard Webb, MD.
16
Diagnostic Criteria for IPF Without a Surgical
Lung Biopsy
Updated!
  • All major criteria and at least 3 minor criteria
    must be present to increase the likelihood of an
    IPF diagnosis
  • Criteria currently under revision (2008)

Not included in current guidelines ATS/ERS. Am
J Respir Crit Care Med. 2000161646-664.
17
Distinguishing IPF From NSIP
Updated!
Lynch DA, et al. Radiology. 200523610-21. Travis
WD, et al. Am J Respir Crit Care Med.
20081771338.
18
Radiologic Diagnosis Inconclusive
Subpleural reticular opacities
Both scans show subpleural reticulation. This
appearance may represent UIP/IPF or fibrotic
NSIP. Biopsy is needed for differentiation.
Images courtesy of W. Richard Webb, MD.
19
Histopathology Resolves Diagnosis
Peripheral Fibrosis
Diffuse Alveolar Wall Fibrosis
Normal Lung
UIP
NSIP
Images courtesy of Kevin O. Leslie, MD.
20
Utility of Lung Biopsy for Diagnosis of IPF
Suspected IPF n 91
Confident diagnosis of IPF n 46 (51)
No confident diagnosis of IPF n 45 (49)
UIP by SLB n 37 (80)
Not UIP by SLB n 9 (20)
UIP by SLB n 17 (38)
Not UIP by SLB n 28 (62)
Hunninghake GW, et al. Am J Respir Crit Care Med.
2001164193-196.
21
Video-Assisted Thoracic Surgery (VATS)
  • High diagnostic accuracy
  • Less morbidity and mortality than open lung
    biopsy
  • Ideal biopsy
  • Two or more surgical wedge biopsies with areas of
    normal lung taken from different areas of the
    lung
  • Samples should measure 35 cm in length and 23
    cm in depth
  • Outpatient thoracoscopic lung biopsy in patients
    with interstitial or focal lung disease
  • Diagnosis obtained in 61/62 patients
  • 72.5 discharged home within 8 hours
  • 22.5 discharged home within 23 hours

Rena O, et al. Eur J Cardiothorac Surg.
199916624-627. Chang AC, et al. Ann Thorac
Surg. 2002741942-1946.
22
Pathological Sections Demonstrating UIP
b. Transition into uninvolved lung
a. Peripheral accentuation of disease
Fibrosis
Normal lung
Fibroblast focus
Normal lung
Normal lung
d. High power image of fibroblastic foci
c. Microscopic honeycombing
Myofibroblasts
Chronic inflammation
Minimal chronic inflammation
Mucus-filled cysts
Courtesy of Kevin O. Leslie, MD.
Courtesy of Kevin O. Leslie, MD.
Pleura
Images courtesy of Kevin O. Leslie, MD.
23
Early/Low Burden Pathology
UIP pattern
Images courtesy of W. Richard Webb, MD, and Kevin
O. Leslie, MD.
24
Classic IPF Pathology
UIP pattern
Images courtesy of W. Richard Webb, MD, and Kevin
O. Leslie, MD.
25
Advanced IPF Pathology
Advanced honeycomb lung more often present
UIP pattern
Images courtesy of W. Richard Webb, MD, and Kevin
O. Leslie, MD.
26
Chronic Hypersensitivity Pneumonitis HRCT
Image courtesy of W. Richard Webb, MD.
27
Chronic Hypersensitivity Pneumonitis
Histopathology
Image courtesy of Kevin O. Leslie, MD.
28
Distinguishing IPF From HP
Image courtesy of Kevin O. Leslie, MD.
29
Subacute Hypersensitivity Pneumonitis HRCT
Image courtesy of W. Richard Webb, MD.
30
Subacute Hypersensitivity Histopathology
Higher Magnification
Images courtesy of Kevin O. Leslie, MD.
31
Scleroderma HRCT
Image courtesy of W. Richard Webb, MD.
32
Scleroderma NSIP
Image A
Image B
Images courtesy of Kevin O. Leslie, MD.
33
Take Home Messages
Updated!
  • Typical clinical features include age gt 50 years,
    gender (male gt female), insidious onset of
    dyspnea, nonproductive cough, and bibasilar
    crackles
  • Atypical clinical or radiologic findings in a
    patient with suspected IPF should prompt the
    clinician to consider obtaining a surgical lung
    biopsy
  • IPF has characteristic UIP histologic features
    that enable definitive diagnosis when clinical
    and radiologic findings are not conclusive
  • Follow-up HRCT is not recommended except with
    significant clinical change
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