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Continued Improvements in IPF Diagnosis: Effective Pathways Toward Accurate Diagnosis

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Assess the impact of a multidisciplinary approach to diagnosis ... Associated with a histologic pattern of usual interstitial pneumonia (UIP) ... – PowerPoint PPT presentation

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Title: Continued Improvements in IPF Diagnosis: Effective Pathways Toward Accurate Diagnosis


1
Continued Improvements in IPF Diagnosis
Effective Pathways Toward Accurate Diagnosis
2
Learning Objectives
  • Utilize the knowledge gained to formulate
    practical diagnostic strategies that foster
    multidisciplinary collaboration and enhance
    diagnostic accuracy
  • Assess the impact of a multidisciplinary approach
    to diagnosis
  • Identify the ATS/ERS diagnostic criteria for IPF
  • Distinguish between typical and atypical IPF
    features on HRCT

3
Current Definition of IPF
  • A distinct type of chronic fibrosing interstitial
    pneumonia
  • Unknown cause
  • Limited to the lungs
  • Associated with a histologic pattern of usual
    interstitial pneumonia (UIP)

ATS/ERS Consensus Statement. Am J Respir Crit
Care Med. 2002165277-304.
4
Importance of Early Diagnosis of IPF
  • Excludes other more treatable diseases
  • Facilitates earlier referral and enrollment in
    clinical trials
  • Generally limited to patients with mild to
    moderate disease
  • Preliminary evidence suggests potential
    improvement in therapeutic response
  • Earlier evaluation for lung transplant
  • Emerging recognition of rapid fatal deterioration
    as a risk even among patients with mild
    impairment

5
Diagnostic Criteria for IPF in the Absence of a
Surgical Lung Biopsy
All major criteria and at least 3 minor criteria
must be present to increase the likelihood of an
IPF diagnosis
ATS/ERS. Am J Respir Crit Care Med.
2000161646-664.
6
Useful Serologic Tests for the Differential
Diagnosis of Interstitial Lung Disease
  • ESR
  • ANCA
  • ACE
  • Granulomatous

ANA RF Scl-70 antibody Jo-1 antibody
Connective tissue diseases
Hypersensitivity pneumonitis
Hypersensitivity panel (follow up with exposure
history)
IPF
None of the above
ATS/ERS. Am J Respir Crit Care Med.
2000161646-664.
7
Differential Diagnosis of IPF
Diffuse Parenchymal Lung Disease (DPLD)
Idiopathic interstitial pneumonias
DPLD of known cause, eg, drugs or association,
eg, collagen vascular disease
Granulomatous DPLD, eg, sarcoidosis
Other forms of DPLD, eg, LAM, HX, etc
Idiopathic pulmonary fibrosis
IIP other than idiopathic pulmonary fibrosis
Respiratory bronchiolitis interstitial lung
disease
Desquamative interstitial pneumonia
Cryptogenic organizing pneumonia
Acute interstitial pneumonia
Lymphocytic interstitial pneumonia
Nonspecific interstitial pneumonia (provisional)
ATS/ERS Consensus Statement. Am J Respir Crit
Care Med. 2002165277-304.
8
Diagnostic Process in DPLD
History, physical examination, lung function
tests, chest radiograph
Not IIP
HRCT
eg, associated collagen vascular disease,
environmental, drug related, etc
Possible IIP
Suspected
Atypical clinical or CT features for IPF
Confident CT diagnosis of IPF with
consistent clinical features and BAL/- TBB that
excludes other disease
other DPLD
If nondiagnostic
TBBx, BAL, or
TBBx or BAL?
other relevant test
Surgical lung biopsy
Non-IIP confirmed
IPF
DIP
NSIP
LIP
RB-ILD
COP
AIP
Modified from ATS/ERS. Am J Respir Crit Care
Med. 2002165277-304.
9
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 A-aPO2 gradient

ATS/ERS. Am J Respir Crit Care Med.
2000161646-664.
10
Clinical Features Are Not Specific
NS Not significant
Flaherty KR, et al. Eur Respir J. 200219275-283.
11
Chest Radiographic Findings
IPF cannot be ruled out by a normal chest x-ray
16 of patients with ILD have normal chest x-rays
Orens JB, et al. Chest. 1995108109-115.
12
Chest Radiograph
Courtesy of W. Richard Webb, MD.
13
Classic IPF HRCT
Courtesy of W. Richard Webb, MD.
14
Definite Advanced IPF
Courtesy of W. Richard Webb, MD.
15
Early/Low Burden IPF
Courtesy of W. Richard Webb, MD.
16
HRCT
Prone scans are often best for showing early
abnormalities
ATS/ERS. Am J Respir Crit Care Med.
2000161646-664.
17
IPF Diagnosis HRCT
Hunninghake GW, et al. Am J Respir Crit Care Med.
2001164193-196.
18
Utility of Lung Biopsy for the 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.
19
Probability of Histologic Diagnosis of Diffuse
Diseases
Transbronchial Biopsy
Surgical Biopsy
1. Granulomatous diseases
2. Malignant tumors/lymphangitic
Often
3. Eosinophilic pneumonia
4. Certain infections
5. Alveolar proteinosis
6. DAD (any cause)
7. Vasculitis
Sometimes
8. Amyloidosis
9. EG/HX/PLCH
10. LAM
11. RB/RB-ILD/DIP
12. UIP/NSIP/LIP COP
13. Small airways disease
14. PHT and PVOD
Courtesy of Kevin O. Leslie, MD.
20
Video-Assisted Thoracic Surgery (VATS)
  • High diagnostic accuracy
  • Less morbidity and mortality than surgical 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

Chang AC, et al. Ann Thorac Surg.
2002741942-1946.
Rena O, et al. Eur J Cardiothorac Surg.
199916624-627.
21
Pathological Sections Demonstrating UIP
b. Transition into uninvolved lung
a. Peripheral accentuation of disease
d. High power image of fibroblastic foci
c. Low power image of fibroblast foci
Courtesy of Kevin O. Leslie, MD.
Courtesy of Kevin O. Leslie, MD.
22
Nonspecific Interstitial Pneumonia Classic
  • Temporal uniformity on biopsy
  • No/few fibroblastic foci
  • Fine reticulation
  • Ground-glass opacities

ATS/ERS. Am J Respir Crit Care Med.
2002165277-304.
23
Multidisciplinary Approach
Multidisciplinary interactions improve diagnostic
agreement and confidence
Modified from Flaherty KR, et al. Am J Respir
Crit Care Med. 2004170904-910.
24
Take Home Messages
  • Typical findings on clinical and radiologic exams
    may preclude the need for a surgical lung biopsy
  • Early diagnosis may confer important benefits in
    patients with IPF
  • Multidisciplinary collaboration enhances
    diagnostic agreement
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