UIP/IPF - Radiology CME

Transcription

David P. Naidich, MD: Usual interstitial
pneumonia (UIP/IPF) vs nonspecific interstitial
pneumonia (NSIP): can we tell the difference on
HRCT?
May 15, 2008- 9:30 AM
Idiopathic Pneumonia: ATS/ERS Classification
Idiopathic Pulmonary Fibrosis (IPF)
vs Non
Non--Specific Interstitial
Pneumonitis (NSIP):
Can We Tell the Difference
Clinical
Syndrome
Imaging Findings
Diff Dx
IPF
Peripheral basilar reticulation; traction
bronchiectasis; honeycomb lung
CVD, drugs,
asbestos
NSIP
Groundglass attenuation; peripheral
reticulation; traction bronchiectasis
CVD, drugs
AIP
Diffuse GG attenuation/consolidation
ARDS
RB/ RB-ILD
Centrilobular GG nodules; patchy GG
attenuation; mild reticulation
Smoking
DIP
Diffuse GG attenuation
Smoking
OP (BOOP)
GG attenuation/consolidation; basilar
distribution (common)
CVD, drugs,
infection, fumes
LIP
Centrilobular GG nodules/ cysts
Aids
Clinical
Syndrome
Imaging Findings
Diff Dx
IPF
CVD, drugs,
asbestos
NSIP
GG attenuation; peripheral reticulation;
traction bronchiectasis
CVD, drugs
AIP
ARDS
RB/ RB-ILD
Smoking
DIP
Smoking
OP (BOOP)
CVD, drugs,
infection, fumes
LIP
Aids
Idiopathic Pulmonary Fibrosis (IPF)
• Clinical Findings:
– Mean age 57; insidious onset; mortality rate – 60-70%; mean
survival 5 – 6 yrs; no response to steroids
• Pathologic Findings:
– Heterogeneous temporal appearance; fibrogenic foci
di i i features;
distinctive
f
fibroblastic
fib bl i proliferation
lif
i with
i h scant
interstitial inflammation; microscopic leading to
gross honeycombing
• CT Findings:
– Honeycombing (+++); peripheral reticulation (++);
groundglass (+); consolidation (+); lower lobe
predominance (+++)
Ellis S. Eur Radiol 2002;12
Usual Interstitial
Pneumonia (UIP)
• temporal and spatial
inhomogeneity
• normal lung
g
• alveolar wall thickening
and inflammation
• fibrosis
(fibroblastic foci)
Courtesy: Ric Webb, MD
Stanford Radiology 10th Annual Multidetector
CT Symposium
1
HRCT?
May 15, 2008- 9:30 AM
UIP
(IPF)
.
2 yrs later
IPF (UIP): CTCT-Pathologic Correlations
• Prospective, multi-center study of 91 pts suspected of having
IPF with clinical, physiologic, CXR, and CT features
assessed by 4 radiologists with F/U histologic correlation
– Analyzed with uni- and multivariate logistic regression
analysis to compare pts with and without documented IPF
• 54 (59%) of 91 pts pathologic dx IPF.
• Using multivariate analysis of specific HRCT features - only
independent predictors of IPF included:
– Honeycombing (odds ratio, 5.36) and upper lobe
reticulation lung (odds ratio, 6.28)
• Using these features specific Dx of IPF established:
– Sens = 74%; Spec = 81%; PPV = 85%.
Hunninghake G. Chest 2003;124:1215-1223
CT Symposium
IPF: Spectrum of HRCT Findings
• Groundglass predominance
• Asymmetric disease
• Focal air-trapping
– mimics
cs cchronic
o c hypersensitivity
ype se s t v ty pneumonitis
p eu o t s
• Pulmonary ossification
• Mediastinal/hilar adenopathy
• Acute exacerbation
– rapid development of diffuse alveolar damage (DAD)
• TB and Lung cancer
Souza C. AJR 2005;185
2
HRCT?
Do HRCT Findings of UIP Obviate Biopsy?
Pulmonologist’s Views
• Questionnaire members of the ACCP whether
HRCT could replace lung bx in 16 DILD’s
• 67% of 230 responders accepted HRCT dx of
IPF/UIP despite awareness of guidelines
recommending histologic diagnosis
• Majority would not accept HRCT diagnosis for
most other diseases even when HRCT is
characteristic.
– LAM (37%) or LCH (19%)
Diette GB. Respiration 2005;72:127-8
Clinical
Syndrome
Imaging Findings
Diff Dx
IPF
CVD, drugs,
asbestos
NSIP
Groundglass attenuation; peripheral
reticulation; traction bronchiectasis
CVD, drugs
AIP
ARDS
RB/ RB-ILD
Smoking
DIP
Smoking
OP (BOOP)
CVD, drugs,
infection, fumes
LIP
Aids
May 15, 2008- 9:30 AM
IPF: Consensus Statement
American Thoracic Society
Major Criteria
Minor Criteria
– Exclusion of other known
etiologies
– Abnormal PFT
PFT’S
S
– Abn HRCT > 6 mos
– TBBX/BAL excluding
other etiologies
– Age > 50 yrs
– Insidious onset DOE
– Diagnosis > 3 mos
duration
– Bibasilar rales
In absence of OLB: Dx requires all 4 major - 3/4 minor
criteria
Non--Specific Interstitial Pneumonitis
Non
• Clinical Findings:
– Broad age range; dyspnea, cough and fever without clubbing:
60% idiopathic - minimum 5% mortality. Associated with
autoimmune diseases (Hasimoto’s thyroiditis; drug reaction) and
collagen vascular disease
• Pathologic Findings: 2 Forms
– Cellular NSIP: homogeneous changes without fibrogenic foci
foci,
alveolar macrophages, or architectural distortion/honeycombing.
Variable degrees of interstitial inflammation and fibrosis
– Fibrotic NSIP: diffuse fibrosis +/- honeycombing
• CT Findings:
– Predominantly GG attenuation admixed with reticulation of
variable severity “often sparing portions of the subpleural lung”
without honeycombing
Nonspecific Interstitial
Pneumonia (NSIP)
• temporal and spatial
homogeneity
• mild interstitial
pneumonia with cellular
infiltration
• cellular or fibrotic forms
• fibrosis relatively mild
Courtesy of Kevin Leslie
CT Symposium
3
HRCT?
May 15, 2008- 9:30 AM
NSIP
NSIP
NSIP
CT Symposium
GGO;
mild reticulation;
Cellular NSIP:
Response to treatment
4
HRCT?
May 15, 2008- 9:30 AM
NSIP
Post Steroids
UIP/IPF
Reticular markings,
Groundglass
Patchy distribution
Honeycombing
y
g
Subpleural sparing
Subpleural dx
Basilar predominance
Fibrotic NSIP:
Scleroderma
IPF
IPF vs NSIP: Diagnostic Accuracy HRCT
IPF
• Compared CT findings NSIP (n = 21) with UIP (n = 32)
• CT diagnosis of NSIP: Sensitivity = 70%; Specificity = 63%
CT diagnosis of IPF: Sensitivity = 63%; Specificity = 70%
• Predominance of groundglass attenuation the cardinal feature of
NSIP
– (odds ratio: 1.04
1 04 for each 1% increase in proportion of GG
attenuation)
• Of patients presenting with clinical diagnosis IPF – NSIP correctly
diagnosed in 70%
• 33% of pts with IPF had equivalent extent of GG attenuation and
reticulation; 12% had predominant GG attenuation!
• Conclusion: Considerable overlap exists IPF vs NSIP
MacDonlad S. Radiology 2001;221
CT Symposium
5
HRCT?
NSIP: CT – Pathologic Subgroup Correlations
• 55 cases documented NSIP categorized into 4 grades:
– Grade 1 - inflammation to Grade 4 - predominant fibrosis
• CT Findings:
– Areas of groundglass attenuation and architectural distortion
identified in all cases.
– Traction bronchiectasis seen in 94%; intralobular reticular
opacities seen in 87%
– Extent of traction bronchiectasis and reticulation correlated with
the histologic grade (p < .001 and .05, respectively)
– Honeycombing identified in 12 (43%) of 28 pts with grade 4
disease and 3 (11%) of the remaining 27 pts (p < .001)
• Conclusion: Extent and severity of bronchiectasis and reticulation
increase with worsening fibrosis
Johkoh T. Radiology 2002;225
May 15, 2008- 9:30 AM
IPF (UIP) VS Chronic IIP
Sumikawa H. Radiology 2006;241:258
• Retrospective study 92 path proved cases IIP:
– UIP=20; cellular NSIP=16; fibrotic NSIP=16; RB-ILD=11; DIP=15; LIP=14.
Excluded AIP and OP. Pts with classic CT findings UIP not biopsied
• Overall correct diagnosis 145 (79%) of 184 reads
• IPF correctly dxed – 63% of cases
– 9/15 cases with IPF - misdiagnosed as NSIP (minimal
or no honeycombing)
– 5 cases with NSIP – misdiagnosed as IPF (3/5
honeycombing and peripheral reticulation
• No statistical difference in extent of GGA, extent
of traction bronchiectasis or peripheral distribution
–between IPF and NSIP
NSIP: Extensive GGO/
Mild reticulation
Traction bronchiectasis
CT Symposium
6
HRCT?
May 15, 2008- 9:30 AM
NSIP and IPF: Changes in Pattern Over Time
Fibrotic
NSIP
Silva C. Radiology 2008;247:2512008;247:251-259
• Retrospective study 48 pts – 23 NSIP; 25 IPF followed
for 34 – 155 months
• HRCT findings reviewed – pattern and distribution:
– groundglass attenuation (GGA); reticulation; traction
b
bronchiectasis;
hi t i honeycombing
h
bi
• NSIP initially correctly identified 18 (78%) of 23 pts
• 5 (28%) of 18 follow-up HRCT more consistent IPF with
marked decrease GGA; increase reticulation and
likelihood of peripheral distribution (p < .05)
• No significant differences predictive of pattern of
evolution based on initial HRCT appearances
NSIP: Report of an ATS Project
Non Specific Interstitial Pneumonitis
• Is NSIP a separate clinical entity?
– or an early form of UIP?
•
•
•
•
•
If so: what are its characteristics?
Clinical findings
Radiologic/ HRCT appearances
Pathologic characteristics: and
How to establish the diagnosis?
– vs. UIP/IPF and chronic hypersensitivity
pneumonitis
Travis W.D. Am J Resp Crit Care Med 2008 (in press)
• Multidisciplinary study (pathology/radiology/clinical)
• 67 cases identified as idiopathic NSIP of 193 reviewed
• Cases a-priori excluded if known etiology:
– collagen vascular disease; drug exposure; airborne
antigens
i
• 126 cases excluded – including 80 possible NSIP; 46
definitively not NSIP
– 3 major groups – HP, UIP, Organizing Pneumonia
• Conclusion: NSIP is a distinct clinical entity occurring
most often in middle aged, non-smoking women (67%);
good prognosis
• HRCT findings: Most common
– lower lung zone involvement (92%); reticular pattern (87%);
traction bronchiectasis (82%); volume loss (77%)
• HRCT findings: Less common
• HRCT findings: Most common
– lower lung zone involvement (92%); reticular pattern (87%);
traction bronchiectasis (82%); volume loss (77%)
• HRCT findings: Less common
– peripheral distribution (46%); Groundglass attenuation (44%);
subpleural sparing (21%) and peribronchial thickening (6.6%)
– Honeycombing identified in 4.9% only
– peripheral distribution(46%); Groundglass attenuation (44%);
subpleural sparing (21%) and peribrochial thickening (6.6%)
– Honeycombing identified in 4.9% only
• Pathologic diagnosis made in 104 cases (definite = 31;
probable = 74): of these, consensus dx other than NSIP
reached in 38 (37%) of cases –36 due to atypical HRCT
findings - most often a pattern of subacute HP or COP.
• Pathologic diagnosis made in 104 cases (definite = 31;
probable = 74): of these, consensus dx other than NSIP
reached in 38 (37%) of cases –36 due to atypical HRCT
findings - most often a pattern of subacute HP or COP.
CT Symposium
7
HRCT?
May 15, 2008- 9:30 AM
DILD: Diagnostic Algorithm
Hx, Physical; CXR; PFT’s, HRCT, BAL
DILD Known
Etiology:
Drugs; CVD
Idiopathic
interstitial
pneumonia
Definite UIP/IPF
Granulomatous
disease:
Sarcoid; HP
Miscellaneous:
diseases
Chronic HP;EG
Surgical biopsy in absence of
confident diagnosis IPF
NSIP; COP (BOOP); AIP;
RB-ILD; DIP; LIP
Adapted: Bhalla M.
JTI 2003
DILD Known
Etiology:
Drugs; CVD
Idiopathic
interstitial
pneumonia
Granulomatous
disease:
Sarcoid; HP
Miscellaneous:
diseases
Chronic HP;EG
Definite Diagnosis
UIP/IPF
(60% Clinical + HRCT)
RB-ILD; DIP; LIP
Adapted: Bhalla M.
JTI 2003
DILD Known
Etiology:
Drugs; CVD
Idiopathic
interstitial
pneumonia
Clinical; HRCT
Diagnosis + IPF
Adapted: Bhalla M.
JTI 2003
Granulomatous
disease:
Sarcoid; HP
Miscellaneous:
diseases
Chronic HP;EG
RB-ILD; DIP; LIP
DILD Known
Etiology:
Drugs; CVD
Idiopathic
interstitial
pneumonia
Clinical; HRCT
Diagnosis + IPF
Adapted: Bhalla M.
JTI 2003
Granulomatous
disease:
Sarcoid; HP
Miscellaneous:
diseases
Chronic HP;EG
Where Specific Dx of NSIP
requires Path-HRCT correlation
Cellular vs Fibrosing IIP’s and Prognosis
Cellular vs Fibrosing IIP’s and Prognosis
Churg A, Muller N. Chest 2006;130:15662006;130:1566-1570
• Good Prognosis:
– Path - Pure cellular process/ +/- Organizing pneumonia
– HRCT- GG attenuation (GGA)/airspace consolidation
• Intermediate Prognosis
– Path – linear fibrosis (follows alveolar walls without
architectural distortion
– HRCT- GGA/ consolidation + reticulation (< 25%)
• Poor Prognosis
– Path – UIP fibrosis/architectural
distortion/microscopic honeycomb
– HRCT- extensive reticulation/honeycombing
CT Symposium
• Good Prognosis:
– Cellular NSIP, RBILD-most DIP, Subacute HP,
COP/BOOP, cellular drug reactions/CVD
g
– Fibrotic NSIP (including NSIP-like HP and CVD
– Some cases DIP
• Poor Prognosis
– UIP (including UIP – like HP, CVD and drug reaction)
– Rare DIP
Adds HP; Excludes AIP and LIP
8
HRCT?
Chronic HP: CT Features – Comparison with
Pathologic Evidence of Fibrosis and Survival
• Retrospective study – 26 pts – open lung bx
– Data base 4000 pts – 1982-2004
• 15 pts – histologic evidence of fibrosis
• These
Th
more likely
lik l to
t have:
h
– Traction bronchiectasis (p = .015)
– honeycombing (p = .007) and
– UIP pattern on CT (p = .007)
(subpleural reticulation/lower zone predominance)
Sahin H. Radiology 2007;244:5912007;244:591-598
May 15, 2008- 9:30 AM
Cellular vs Fibrosing IIP’s: Diagnostic Approach
• Good Prognosis: =
TBBx/BAL - Empirical Rx
– Path - Pure cellular process/ +/- Organizing pneumonia
– HRCT- GG attenuation (GGA)/airspace consolidation
g
=
Surgical Lung Bx
– Path – linear fibrosis (follows alveolar walls without
architectural distortion
– HRCT- GGA/ consolidation + reticulation (< 25%)
• Poor Prognosis =
Conservative Management
– Path – UIP fibrosis/architectural distortion/microscopic
honeycomb
– HRCT- extensive reticulation/honeycombing
HRCT Diagnosis of IPF
• Basal honeycombing visible on HRCT has a
high predictive value for UIP
• In the absence of a known disease or
exposure,
IPF is very likely the diagnosis
• Lung biopsy is unlikely to be performed
• Poor prognosis; treatment of little value
• Not all IPF shows honeycombing
CT Symposium
9

UIP/IPF - Radiology CME

Transcription

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