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Early and Accurate Diagnosis

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Early and Accurate Diagnosis of Idiopathic Pulmonary Fibrosis (IPF) Desaturators Lama VN, et al. Am J Respir Crit Care Med. 2003;168:1084-1090. – PowerPoint PPT presentation

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Title: Early and Accurate Diagnosis


1
Early and Accurate Diagnosis of Idiopathic
Pulmonary Fibrosis (IPF)
2
Objective
Discuss the diagnostic strategies for
facilitatingearly and accurate diagnosis for IPF
(clinical,radiologic, and pathologic evaluations)
3
Approach to Diagnosis of IPF
  • Clinical evaluation
  • Radiologic evaluation
  • Pathologic evaluation

4
Clinical Evaluation
  • Major criteria
  • Exclusion of other known causes of ILD
  • Abnormal pulmonary function tests that include
  • evidence of restriction and impaired gas
    exchange
  • Bibasilar reticular abnormalities with minimal
    ground
  • glass opacities on HRCT scan
  • Transbronchial lung biopsy or BAL without
    features
  • to support an alternative diagnosis

ATS/ERS Consensus Statement. Am J Respir Crit
Care Med. 2002165277-304.
5
Clinical Evaluation
  • Minor criteria
  • Age gt 50 yr
  • Insidious onset of otherwise unexplained
  • dyspnea on exertion
  • Duration of illness gt 3 mo
  • Bibasilar, inspiratory Velcro-like crackles
  • All major criteria and at least 3 of the minor
    criteria must be
  • present to increase the likelihood of an IPF
    diagnosis

ATS/ERS Consensus Statement. Am J Respir Crit
Care Med. 2002165277-304.
6
Pulmonary Function Tests
  • Typical findings in IPF patients
  • Restriction
  • Reduced FVC and TLC
  • Normal or increased FEV1/FVC ratio
  • Impaired gas exchange
  • Decreased DLco, PaO2
  • Desaturation on exercise oximetry
  • Increased A-a gradient
  • Normal resting PFTs do not exclude IPF

ATS/ERS Consensus Statement. Am J Respir Crit
Care Med. 2000161646-664.
7
6-Minute Walk Test for
Interstitial Lung Disease
  • Baseline blood pressure, pulse, O2 saturation
  • Timed walk at any pace
  • As many stops as necessary
  • If oxygen saturation persistently less than
  • 88, repeat test with supplemental oxygen
  • Primary endpoint is walk distance

Enright PL. Respir Care. 200348783-785 ATS. Am
J Respir Crit Care Med. 2002166111-117.
8
Desaturation on 6-Minute Walk Test
  • Desaturation may occur in several lung diseases
  • Interstitial lung diseases
  • Pulmonary hypertension
  • Severe obstructive lung disease
  • If desaturation occurs on 6MWT and an ILD is
  • suspected, then HRCT may be indicated

Enright PL. Respir Care. 200348783-785 ATS. Am
J Respir Crit Care Med. 2002166111-117.
9
Desaturators
Desaturation on 6MWT Predicts Decreased Survival
in UIP (N 83)
1.0
69
0.8
0.6
35
Survival Probability
0.4
P 0.0018
0.2
0.0
0
2
1
3
4
5
Nondesaturators
Years
  • Lama VN, et al. Am J Respir Crit Care Med.
    20031681084-1090.

10
Desaturation on 6MWT Predicts Decreased Survival
in NSIP (N 22)
100
1.0
0.8
66
0.6
Survival Probability
0.4
P 0.0089
0.2
0.0
0
2
1
3
4
5
Nondesaturators
Years
Desaturators
Lama VN, et al. Am J Respir Crit Care Med.
20031681084-1090.
11
Differential Diagnosis
  • Other idiopathic interstitial pneumonias
  • NSIP
  • DIP
  • RBILD
  • AIP
  • COP
  • Connective tissue diseases
  • Scleroderma
  • Rheumatoid arthritis
  • Polymyositis/dermatomyositis
  • Asbestosis
  • Hypersensitivity pneumonitis
  • Sarcoidosis

ATS/ERS Consensus Statement. Am J Respir Crit
Care Med. 2000161646-664.
12
Diseases That Mimic IPF
  • IPF is often misdiagnosed, or diagnosed at an
    advanced stage of the disease
  • Symptoms of other diseases that mimic IPF
  • COPD
  • CHF
  • Connective tissue diseases (eg, RA, Sjögrens,
    SLE)
  • Other lung diseases (asbestosis,
    hypersensitivity
  • pneumonitis)
  • ATS/ERS Consensus Statement. Am J Respir Crit
    Care Med. 2000161646-664.
  • ATS/ERS Consensus Statement. Am J Respir Crit
    Care Med. 2002165277-304.

13
Clinical Evaluation
  • Useful serology to exclude/include other diseases
  • ESR
  • ANA
  • RF
  • Scl-70
  • CPK and aldolase
  • Jo-1 antibody
  • ANCA
  • ACE
  • Hypersensitivity panel (follow-up with exposure
    history)

Adapted from ATS/ERS Consensus Statement. Am J
Respir Crit Care Med. 2000161646-664.
14
Idiopathic Pulmonary Fibrosis (IPF)Chest X-Ray
Findings
  • Reduced lung volume
  • Reticular opacities
  • Honeycombing sometimes visible
  • Lower-lobe and peripheral predominance of
  • abnormalities
  • HRCT should be performed in all patients with
  • suspected IPF, even if CXR is typical

15
Typical Features of IPF on Chest X-Ray
Abnormal CXR
Normal CXR
Slide courtesy of Ganesh Raghu, MD.
16
Typical Features of IPF on Chest X-Ray (cont.)
17
  • Typical Features of IPF on Chest X-Ray (cont.)

18
High-Resolution CT Technique
  • Thin collimation (eg, 1 mm)
  • Spaced images (12 cm) at full inspiration
  • Volumetric helical HRCT may be used but results
    in a greater radiation dose
  • Additional prone scans often best for showing
    early
  • abnormalities
  • Window mean -600 to -700 H
  • Window width 1000 to 1500 H
  • Slide courtesy of W. Richard Webb, MD.

19
Idiopathic Pulmonary Fibrosis (IPF) HRCT
Findings
  • Peripheral honeycombing
  • Irregular reticular opacities
  • Traction bronchiectasis
  • Minimal ground-glass opacity
  • Sub-pleural, posterior, lower-lobe
    predominance of
  • abnormalities
  • ATS/ERS Consensus Statement. Am J Respir Crit
    Care Med. 2000161646-664.

20
Early HRCT Findings in IPF
Slide courtesy of Kevin O. Leslie, MD.
21
UIP Honeycombing
Slide courtesy of W. Richard Webb, MD.
22
UIP Minimal Honeycombing
Slide courtesy of W. Richard Webb, MD.
23
UIP Irregular Reticular Opacities
Slide courtesy of W. Richard Webb, MD.
24
UIP Traction Bronchiectasis
Slide courtesy of W. Richard Webb, MD.
25
Subpleural and Basal Predominance
Slide courtesy of W. Richard Webb, MD.
26
HRCT Diagnosis of IPF
  • Prospective study of 91 patients with
    suspected IIP
  • 54 (59) had IPF
  • 37 (41) had other diseases
  • On HRCT, radiologists certain of dx in 52
    (57) of 91
  • Certain IPF diagnosis correct in 26 (96) of
    27
  • Certain non-IPF correct in 21 (84) of 25
  • Confident clinical/HRCT diagnosis of IPF
    SLB probably
  • unnecessary
  • Confident clinical/HRCT not IPF and
    diagnosis uncertain
  • SLB indicated

Hunninghake et al. Am J Respir Crit Care Med
2001 164193-196.
27
HRCT Diagnosis of IPF
  • Independent predictors of IPF
  • Lower-lobe honeycombing (odds ratio 5.36 P
    0.007)
  • Upper-lobe reticular opacities (odds ratio 6.28
    P 0.004)
  • In patients presenting with a clinical
    syndrome suggestive of IIP, CT findings of
    lower-lung honeycombing and upper-lung
    irregular lines are most closely associated with
    a pathologic diagnosis of UIP

Hunninghake et al. Chest 2003 1241215-1223.
28
HRCT Diagnosis of IPF
Diagnosis Sensitivity Specificity
HRCT consistent with IPF 80 84
Radiograph consistent with IPF 89 75
HRCT or radiograph consistent with IPF 91 72
HRCT and radiograph consistent with IPF 78 87
Hunninghake et al. Chest 2003 1241215-1223.
29
HRCT Diagnosis of IPF
  • 73 patients with UIP 23 with NSIP
  • Honeycombing on HRCT definite or probable
    UIP
  • All 27 patients with a HRCT diagnosis of
    definite or probable UIP had UIP on biopsy
  • Patients with HRCT diagnosed definite or
    probable UIP had shortest survival

Flaherty et al. Thorax. 200358143-148.
30
Survival in Relation to HRCT and Histology
Deaths/Patients ()
Median Survival (yrs)
Diagnostic Category
34/73 (47) 2/23 (9)
3.98 gt 9
Histological Diagnosis UIP NSIP
17/27 (63) 9/25 (36) 10/44 (23)
2.08 5.76 5.81
HRCT Diagnosis Definite/probable UIP
Indeterminate Definite/probable NSIP
17/27 (63) 17/46 (37) 2/18 (11)
0/5 (0)
2.08 5.76 gt 9 gt 6.6
Histological HRCT UIP UIP
NSIP NSIP
Def/Prob UIP Def/Prob NSIP Def/Prob
NSIP Indeterminate
Table adapted from Flaherty KR, et al. Thorax.
200358(2)143-148.
31
Role of BAL vs Biopsy in IPF
Procedure Role
Brochoalveolar Lavage (BAL) May rule out alternate diagnoses but not diagnostic of IPF
Transbronchial Biopsy (TBB) May rule out alternate diagnoses but not diagnostic of IPF Often abnormal in IPF but does not confirm diagnosis
Video-assisted Thoracoscopic Biopsy (VATS) Preferred technique Provides best tissue samples Excludes other processes that mimic IPF Biopsies should be obtained from more than one lobe of the lung
ATS/ERS Consensus Statement. Am J Respir Crit
Care Med. 2000161646-664
2002165277-304.
32
Diagnostic Pathologic Findings in UIP
  • UIP pattern
  • Fibroblastic foci
  • Temporal heterogeneity
  • Honeycombing
  • Minimal inflammation

ATS/ERS Consensus Statement. Am J Respir Crit
Care Med. 2000161646-664 2002165277-304. .
33
Approach to Diagnosing IPF
Clinical Evaluation History, PE, CXR, PFTs, 6MWT
Not IIP
Potential IIP
HRCT
Diagnostic of IPF or other diffuse lung disease
Diagnosis uncertain
Transbronchial Bx or BAL
Diagnostic
Nondiagnostic
Surgical lung biopsy
IPF
Not IPF
Adapted from ATS/ERS Consensus Statement. Am J
Respir Crit Care Med. 2002165277-304.
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