View
221
Download
0
Category
Tags:
Preview:
Citation preview
Learning Objectives
• Explain 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 diagnosis
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 exposures
Interstitial Lung Diseases
ILD of Known Cause or
Association
Medications
Radiation
Connective Tissue Disease
Vasculitis & DAH
Hypersensitivity Pneumonitis
Pneumoconioses
Idiopathic Interstitial
Pneumonias
Sarcoidosis & Other
Granulomatous Diseases
Other
LAM
Pulmonary LCH
Eosinophilic Pneumonias
Alveolar Proteinosis
Genetic Syndromes
Adapted from: ATS/ERS Guidelines for IIP. AJRCCM. 2002;165:277-304.
Major Idiopathic Interstitial Pneumonias
Category Clinical-Radiologic-Pathologic Diagnosis
Associated Radiographic and/or Pathologic pattern
Chronic fibrosing
IPF UIP
Idiopathic nonspecific interstitial Pneumonia (iNSIP) NSIP
Smoking-related
Respiratory bronchiolitis-ILD (RB-ILD) Respiratory bronchiolitis
Desquamative interstitial pneumonia (DIP) Desquamative interstitial pneumonia
Acute/ subacute
Cryptogenic organizing pneumonia (COP) Organizing pneumonia
Acute interstitial pneumonia (AIP) Diffuse alveolar damage
Travis et al. Am J Respir Crit Care Med. 2013;188:733-748.
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 fibroelastosis
Unclassifiable Unclassifiable IIP Many
Travis et al. Am J Respir Crit Care Med. 2013;188:733-748.
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.
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 2014
‒ Nintedanib (Ofev)‒ Pirfenidone (Esbriet)
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 management
Cum
ulati
ve P
ropo
rtion
Sur
vivi
ng
Time (years)
Higher Mortality Associated With Delays in Accessing Care
Lamas DJ, et al. Am J Respir Crit Care Med. 2011;184:842-847.
Years
Surv
ival
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
ORDefinite/possible UIP pattern on HRCT with a surgical lung
biopsy showing definite/probable UIP
AND
• Median survival = 3.8 years• Factors associated with lower survival
– Age, 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
Incidence of IPF
Risk factors for higher incidence• Age • Male sex • Hispanic
ethnic origin• Geography
Raghu G, et al. Lancet Respir Med. 2014;2(7):566-572.
Highest Medium
Lowest
When Should I Suspect ILD?
Column A
Exertional Dyspnea
Non-productive Cough
Family History of ILD
Column BAbnormal CXR
Crackles
Exertional Desaturation
Spirometry (low FVC) or low DLCO
One from Column A and one from Column B
“ACES”
ILD FeaturesSimilarities 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
– Honeycombing– Ground glass – Distribution of abnormalities
• Histopathology
Pulmonary Function Tests
• Spirometry – Reduced FVC and TLC
– Normal or increased FEV1/FVC ratio
• Restriction often accompanied by some obstruction
• Impaired gas exchange
– Decreased DLCO, PaO2
– Desaturation on exercise oximetry
– Increased A-aPO2 gradient
• Normal PFTs do not exclude ILD– Emphysema + Interstitial Lung Disease
• Infectious• Inhalational• Immunologic• Iatrogenic• Idiopathic• Cardiovascular• Neoplastic
Mnemonic for Diagnosing ILD
What Should I Do if I Suspect ILD?
http://www.pfdoc.org/2014/07/a-pulmonary-fibrosis-primer-for-doctors.html. Accessed August 2014.
Specific diagnosis
Clinical picture
Radiologic pattern (HRCT)
Pathologic pattern
(lung biopsy)
High Resolution CT scan
• Inspiratory 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.
Possible UIP Pattern
Hodnett PA, et al. Am J Respir Crit Care Med. 2013;188:141-149.
traction bronchiectasis
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.
Inconsistent With UIP
Hodnett PA, et al. Am J Respir Crit Care Med. 2013;188:141-149.
distinctlobular pattern
HRCT features inconsistent with IPFInconsistent Features
Upper lobe predominant
Peribronchovascular predominance
Ground-glass > extent of reticular abnormality
Profuse micronodules
Discrete cysts
Diffuse mosaic attenuation/gas-trapping
Consolidation
Raghu G, et al. Am J Respir Crit Care Med. 2011;183:788-824.
What Should I Do if HRCT Confirms ILD?
http://www.pfdoc.org/2014/07/a-pulmonary-fibrosis-primer-for-doctors.html. Accessed August 2014.
Specific diagnosis
Clinical picture
Radiologic pattern (HRCT)
Pathologic pattern
(lung biopsy)
Known Causes of ILD: History & Physical Exam • Drugs
– eg, Amiodarone, bleomycin, nitrofurantoin
– www.pneumotox.com
• Radiation‒ External beam radiation
therapy to thorax
• Connective Tissue Diseases– Rheumatoid arthritis– Systemic sclerosis
(scleroderma)– Idiopathic inflammatory
myopathies– Vasculitis
• Occupational/Environmental– Inorganic antigens
(Pneumoconioses)• Asbestosis• Coal worker’s pneumoconiosis• Silicosis
– Organic antigens (Hypersensitivity Pneumonitis)
• Birds• Mold
Advanced Sclerodactyly
http://images.rheumatology.org. Accessed July 2014.
Serological Evaluation
• Minimum: 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 anticoagulant– Creatine kinase, aldolase– Hypersensitivity pneumonitis panel
• Should be performed before a biopsy
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
ORDefinite/possible UIP pattern on HRCT with a surgical lung
biopsy showing definite/probable UIP
AND
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 center
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
Radi
olog
ic P
atter
n
Summary Diagnosis
Putting it all Together
• Physiology• Full PFTs
• Gas exchange• 6MWT
• Radiology• HRCT
• History• Exam• Labs
• ANA, RF, anti-CCP
• Pathology
Conclusions: Diagnosing IPF
• IPF is a fibrotic ILD• No identifiable cause for fibrosis
–Exposure/CTD are absent
• Either…–Characteristic HRCT pattern–UIP-pattern on surgical lung biopsy
• Multidisciplinary approach enables an accurate diagnosis
Recommended