Diagnosing Idiopathic Pulmonary Fibrosis FACULTY Title Affiliation Learning Objectives Explain the considerations associated with clinical evaluation imaging and biopsy in terms of differentially diagnosing ID: 708211
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NAVIGATING the NEW ERA in IPF:Diagnosing Idiopathic Pulmonary Fibrosis
FACULTY
Title
AffiliationSlide2
Learning ObjectivesExplain the considerations associated with clinical evaluation, imaging, and biopsy, in terms of differentially diagnosing
IPF
Identify opportunities for interdisciplinary
collaboration
and consultation and key aspects of
guideline
recommendations that can facilitate early and accurate IPF
diagnosisSlide3
Interstitial Lung Diseases
Diverse group of disorders that involve the distal pulmonary parenchyma
Typical presentation
Progressive dyspnea and dry cough
Abnormal pulmonary physiology
Abnormal CXR and/or HRCT
Etiology
Idiopathic
Systemic diseases (connective tissue disorders)
Toxic, radiologic, environmental, occupational exposuresSlide4
Adapted from: ATS/ERS Guidelines for IIP.
AJRCCM
. 2002;165:277-304
.Slide5
Major Idiopathic Interstitial Pneumonias
Category
Clinical-Radiologic-Pathologic
Diagnosis
Associated
Radiographic and/or Pathologic pattern
Chronic
fibrosing
IPF
UIPIdiopathic nonspecific interstitial Pneumonia (iNSIP)NSIPSmoking-relatedRespiratory bronchiolitis-ILD (RB-ILD)Respiratory bronchiolitisDesquamative interstitial pneumonia (DIP)Desquamative interstitial pneumoniaAcute/ subacuteCryptogenic organizing pneumonia (COP)Organizing pneumoniaAcute interstitial pneumonia (AIP)Diffuse alveolar damage
Travis et al.
Am J Respir Crit Care Med.
2013;188:733-748.Slide6
Other Idiopathic Interstitial Pneumonias
Category
Clinical-Radiologic-Pathologic
Diagnosis
Associated
Radiographic and/or Pathologic pattern
Rare
Idiopathic lymphoid interstitial pneumonia (iLIP)
Lymphoid interstitial pneumonia
Idiopathic pleuroparenchymal fibroelastosis (IPPFE)Pleuroparenchymal fibroelastosisUnclassifiable Unclassifiable IIPManyTravis et al. Am J Respir Crit Care Med. 2013;188:733-748.Slide7
Diffuse
Parenchymal Lung Disease (DPLD
)
DPLD of known cause, eg, drugs or association, eg, collagen vascular disease
Idiopathic interstitial pneumonias
Granulomatous DPLD, eg, sarcoidosis
Other forms of DPLD, eg, LAM, HX, etc
Idiopathic pulmonary fibrosis
IIP other than idiopathic pulmonary fibrosis
Desquamative interstitial pneumonia
Acute interstitial
pneumonia
Nonspecific interstitial pneumonia (provisional)
Respiratory bronchiolitis interstitial lung disease
Cryptogenic organizing pneumonia
Lymphocytic interstitial pneumonia
Pleuroparenchymal fibroelastosis
Travis WD, et al; ATS/ERS Committee on Idiopathic Interstitial
Pneumonias.
Am
J
Respir
Crit
Care Med
.
2013;188(6
):
733-748
. Slide8
Idiopathic Pulmonary Fibrosis
Normal Lungs
Usual Interstitial PneumoniaSlide9
Idiopathic Pulmonary Fibrosis
Peripheral lobular fibrosis of unknown cause
Clinical impact
Exertional dyspnea
Cough
Functional and exercise limitation
Impaired quality-of-life
Risk for acute respiratory failure and death
Median survival time of 3-5 years
Two new drugs approved by the FDA in October 2014Nintedanib (Ofev)Pirfenidone (Esbriet)Slide10
Parameter
HR (95% CI)
IPF Dx
28.46
(5.5, 147)
Age
0.99 (0.95, 1.03)
Female sex
0.31 (0.13, 0.72)
Smoker
0.30 (0.13, 0.72)
Physio CRP
1.06 (1.01, 1.11)
Onset Sx (yrs)
1.02 (0.93, 1.12)
CTfib score ≥ 2
0.77 (0.29, 2.04)
Diagnosis Matters!
IPF/UIP Confers a Poor Prognosis
Flaherty KR,
et al.
Eur Respir J
. 2002;19:275-283.Correct diagnosis
appropriate managementCumulative Proportion SurvivingTime (years)Slide11
Higher Mortality Associated With Delays in Accessing Care
Lamas DJ, et
al.
Am J Respir Crit Care
Med.
2011
;
184:842-847.
Years
SurvivalSlide12
Raghu
G, et
al
.
Am J Respir Crit Care
Med
. 2011;183:788-824.
2011 ATS/ERS
Diagnostic Criteria for IPF
*also
known as diffuse parenchymal lung disease,
DPLD
Exclusion of
known
causes of ILD*
UIP pattern on HRCT without surgical biopsy
OR
Definite/possible UIP pattern on HRCT with a surgical lung biopsy showing definite/probable
UIPANDSlide13
Idiopathic Pulmonary Fibrosis
Normal Lung
Usual Interstitial PneumoniaSlide14
Idiopathic Pulmonary Fibrosis
Normal Lung
Fibroblastic focus in
Usual
Interstitial PneumoniaSlide15
Median survival = 3.8 yearsFactors associated with lower survivalAge, index year, male sex
Raghu G, et al
.
Lancet
Respir Med
.
2014;2(7
):
566-572
. Prevalence of IPF is IncreasingMedicare Beneficiaries Age ≥ 65 Years Slide16
Incidence of IPFRisk factors for higher incidenceAge
Male
sex
Hispanic ethnic origin
Geography
Raghu G, et al
.
Lancet
Respir Med. 2014;2(7):566-572.
Highest
Medium
LowestSlide17
When Should I Suspect ILD?
One from
Column A
and one from
Column B
“ACES”Slide18
ILD Features
Similarities
Differences
Dyspnea
Progressive
Exertional
Cough
Non-productive
Bibasilar crackles
Restrictive ventilatory defect
Exertional desaturation
ILD on HRCT
Prior/current exposures
Extrapulmonary findings
Sarcoidosis
Connective tissue disease
Joint involvement
Serologies
HRCT
HoneycombingGround glass
Distribution of abnormalities HistopathologySlide19
Pulmonary Function TestsSpirometry Reduced FVC and TLC
Normal or increased FEV
1
/FVC ratio
Restriction often accompanied by some obstruction
Impaired gas exchange
Decreased
DL
CO
, PaO2 Desaturation on exercise oximetry Increased A-aPO2 gradientNormal PFTs do not exclude ILDEmphysema + Interstitial Lung DiseaseSlide20
Infectious
Inhalational
Immunologic
Iatrogenic
Idiopathic
Cardiovascular
Neoplastic
Mnemonic for
Diagnosing ILDSlide21
What Should I Do if I Suspect ILD?http://www.pfdoc.org/2014/07/a-pulmonary-fibrosis-primer-for-doctors.html.
Accessed August 2014.Slide22
High Resolution CT scanInspiratory supine and expiratory supine< 1.25mm axial reconstruction
High spatial frequency reconstruction
(“bone”) algorithm
Prone imaging in select cases
No IV contrast
http://www.pfdoc.org/2013/08/should-i-undergo-lung-biopsy-to.html.
Accessed August 2014.Slide23
UIP Pattern
Hodnett PA, et al.
Am J Respir Crit Care Med.
2013;188:141-149.Slide24
Possible UIP Pattern
Hodnett PA, et al.
Am J Respir Crit Care Med.
2013;188:141-149.
traction bronchiectasisSlide25
HRCT Criteria for UIP
UIP Pattern
Possible
UIP Pattern
Subpleural, basal
predominance
+
+
Reticular abnormality++Honeycombing (+/- traction bronchiectasis)+-Absence of “inconsistent” features++
Raghu
G, et
al
.
Am J Respir Crit Care
Med
. 2011;183:788-824.Slide26
Inconsistent With UIP
Hodnett PA, et al.
Am J Respir Crit Care Med.
2013;188:141-149.
distinct
lobular patternSlide27
HRCT features inconsistent with IPF
Inconsistent
Features
Upper
lobe
predominant
Peribronchovascular
predominanceGround-glass > extent of reticular abnormalityProfuse micronodulesDiscrete cystsDiffuse mosaic attenuation/gas-trappingConsolidation
Raghu
G, et
al
.
Am J Respir Crit Care
Med
. 2011;183:788-824.Slide28
What Should I Do if HRCT Confirms ILD?http://
www.pfdoc.org/2014/07/a-pulmonary-fibrosis-primer-for-doctors.html.
Accessed August 2014.Slide29
Known Causes of ILD:
History & Physical Exam
Drugs
e
g, Amiodarone, bleomycin, nitrofurantoin
www.pneumotox.com
Radiation
External beam radiation therapy to
thorax
Connective Tissue DiseasesRheumatoid arthritisSystemic sclerosis (scleroderma)Idiopathic inflammatory myopathiesVasculitisOccupational/EnvironmentalInorganic antigens (Pneumoconioses)AsbestosisCoal worker’s pneumoconiosisSilicosisOrganic antigens (Hypersensitivity Pneumonitis)BirdsMoldSlide30
Gottron's Papules in Dermatomyositis
http://images.rheumatology.org.
Accessed July 2014.Slide31
Mechanic's Hands in Anti-Synthetase Syndrome
http://images.rheumatology.org.
Accessed July 2014.Slide32
Raynaud's Phenomenon
http://images.rheumatology.org.
Accessed July 2014.Slide33
Puffy Fingers in Early Scleroderma or Mixed CTD
http://images.rheumatology.org.
Accessed July 2014.Slide34
Advanced Sclerodactyly
http://images.rheumatology.org
. Accessed July 2014.Slide35
Digital Clubbing
NEJM, 2001
Reynen K, et al.
N
Engl J
Med.
2000; 343:1235Slide36
Serological EvaluationMinimum: ANA, RF, CCP (ATS/ERS guidelines)Based on history & physical exam, consider:
Extractable nuclear antigen (ENA) autoantibody panel
Anti
-
centromere antibody
ESR & CRP
MPO
/
PR3 (ANCA) antibodies
Anti-cardiolipin antibodies, lupus anticoagulantCreatine kinase, aldolaseHypersensitivity pneumonitis panelShould be performed before a biopsySlide37
Raghu
G, et
al
.
Am J Respir Crit Care
Med
. 2011;183:788-824.
2011 ATS/ERS
Diagnostic Criteria for IPF
*also
known as diffuse parenchymal lung disease,
DPLD
Exclusion of
known
causes of ILD*
UIP pattern on HRCT without surgical biopsy
OR
Definite/possible UIP pattern on HRCT with a surgical lung biopsy showing definite/probable
UIPANDSlide38
Before You Biopsy…
Can you confirm the diagnosis without a biopsy
?
Is it safe?
Extensive honeycombing
Pulmonary hypertension
High oxygen requirements
Progressive disease
Avoid a “diagnostic trial” of steroids if possible
Consider referral to an ILD centerSlide39
Diagnosis of IPF by Lung Biopsy
Raghu
G, et
al
.
Am J Respir Crit Care
Med
. 2011;183:788-824.
UIP
Probable UIP
Possible
UIP
Not UIP
Not performed
UIP
IPF
IPF
IPF
Not IPF
IPF
Possible UIP
IPF
IPF
+/- IPF
Not IPF
Not IPF
Inconsistent with UIP
+/- IPF
Not IPF
Not IPF
Not
IPF
Not IPF
Histopathologic Pattern
Radiologic PatternSlide40
Summary Diagnosis
Putting it all Together
Physiology
Full
PFTs
Gas exchange
6MWT
Radiology
HRCT
HistoryExamLabsANA, RF, anti-CCPPathologySlide41
Conclusions: Diagnosing IPFIPF is a fibrotic ILDNo identifiable cause for fibrosis
Exposure/CTD are
absent
Either…
Characteristic HRCT pattern
UIP-pattern on surgical lung
biopsy
Multidisciplinary approach enables an accurate diagnosisSlide42
QUESTIONS and ANSWERS