Overview of Reproduction Presenter Idiopathic Pulmonary ...
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Overview of Idiopathic Pulmonary Fibrosis: Diagnosis and Therapy Jeff Swigris, DO, MS Director, ILD Program National Jewish Health Property of Presenter Not for Reproduction
Transcript of Overview of Reproduction Presenter Idiopathic Pulmonary ...
Overview of Idiopathic Pulmonary Fibrosis:
Diagnosis and Therapy
Jeff Swigris, DO, MSDirector, ILD ProgramNational Jewish Health
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Disclosures
Speaker - Boehringer Ingelheim and Genentech
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Objectives
Describe how to diagnose IPF
Develop strategy to distinguish IPF from other ILDs
Comprehensive therapeutic approach to IPF
Patient education and taking aim at improving QOL
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ILD
Inflammation and/or scar
In the INTERSTITIUM
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To an ILD doc, the interstitium is located…
Along BV bundles
In interlobular septae
In the subpleural region
Within alveolar walls (largest area)Propert
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Secondary Pulmonary Lobule
~1.5cmPropert
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Etiology-based classification scheme
ILD
Exposure-relatedmold, bacteria, birds
medications XRT dusts
cigarette smoke
IdiopathicSarcoidosis
LAM IIP
GeneticFPF
Autoimmune-relatedRA
Systemic sclerosis PM/DM
Sjögren’s syndrome MCTD SLE IBD
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Idiopathic interstitial pneumonias (IIP)
Major IIP Major IIP Idiopathic pulmonary fibrosis (IPF) Idiopathic pulmonary fibrosis (IPF) Idiopathic nonspecific interstitial pneumonia (NSIP)Idiopathic nonspecific interstitial pneumonia (NSIP) Respiratory Respiratory bronchiolitisbronchiolitis--ILD (RBILD (RB--ILD)ILD) DesquamativeDesquamative interstitial pneumonia (DIP)interstitial pneumonia (DIP) Cryptogenic organizing pneumonia (COP)Cryptogenic organizing pneumonia (COP) Acute interstitial pneumonia (AIP)Acute interstitial pneumonia (AIP)
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ILD ➙ IIP ➙ IPF
IPF: specific type of ILD within the IIP
IPF: specific type of pulmonary fibrosis UIP-pattern
IPF: Diagnosis of exclusion Rule out…
Exposure (environment, Rx, occupational) Connective tissue disease
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Histology-based classification scheme
S C ARFLAMMAT I ON
UIP‐pattern lives here
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Etiology-based classification scheme
ILD
Exposure-relatedmold, bacteria, birds
medications XRT dusts
cigarette smoke
IdiopathicSarcoidosis
LAM IIP
GeneticFPF
Autoimmune-relatedRA
Systemic sclerosis PM/DM
Sjögren’s syndrome MCTD
S C ARNFLAMMAT NI OI
S C ARNFLAMMAT NI OI
S C ARNFLAMMAT NI OI
S C ARNFLAMMAT NI OI
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Idiopathic disease + UIP-pattern
IPF
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What to do when you have a patient in front of you and you
are considering the possibility of IPF as the
clinical summary diagnosis
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Making the diagnosis of IPF You have to be a detective
History
Exam
Pulmonary physiology
Radiography
+/- surgical lung biopsy
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History
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History Typically…IPF presents with:
Dyspnea—subacute, insidious onset
“I thought it was just that I was a year older, 5# heavier, and out of shape”
+/- dry cough Fatigue/low stamina Not pain, swollen joints, rash
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Identify symptoms of rheumatologic illness Eyes/skin/joints/muscles
Family history Pulmonary fibrosis Rheumatologic illness
Exposures Smoking, dusts, M/M/B/B
History
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Physical Exam
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Physical examination
Hands Chest
Crackles ANY VELCRO CRACKLE IS ABNORMAL MAKE SURE YOU LISTEN AT THE BASES
Joints Skin Prop
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Laboratory
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Laboratory assessment
Serologies ANA RF/anti-CCP Scl-70 SSA/SSB Myositis panel
What to do with positive????Prop
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Pulmonary Physiology and Gas Exchange
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Pulmonary physiology
Pulmonary function testing
Measures of resting and exercise-related gas exchange
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Radiology
“ILD protocol” HRCT No IV contrast Supine and prone Inspiratory and expiratory images High-resolution reconstruction algorithm 1-1.5mm thick slicesProp
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Lung Biopsy
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Lung biopsy
Transbronchial biopsy Sarcoidosis Lymphangitic carcinomatosis Subacute HP
Surgical Thorascopic, multiple lobes When “classic” HRCT pattern absent
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Putting it all Together History
Exam
Labs ANA, RF, anti-CCP
Physiology Full PFTs
Gas exchange 6MWT
Radiology HRCT
Pathology
Integrate to get “summary diagnosis”
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Making the diagnosis of IPF
Suspected IPF
Identifiable causes for ILD?
HRCT
Consider VATSx Lung Biopsy
IPF or not IPFIPF Not IPF
Possible UIPIndeterminate UIP
Clin/Rad/Path
UIPProbable/Possible UIPNon-classifiable fibrosis
Yes
Not UIP
No
UIP-pattern
Other Dx
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Park et al. AJRCCM 2007
IPF
CTD
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Ann Intern Med. 2012;156(10):684-691
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IPF: Cause of Death
Olson et al. Am J Respir Crit Care Med 2007
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What’s not IPF?
48 yo F with achey joints, cracked hands, Raynauds
and anti-PL-7 antibodiesProp
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What’s the Diagnosis?
69 yo M with anti-CCP positive RA, now with cough and exertional dyspnea
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What’s not IPF?
69 yo M with parrot, exertional dyspnea and cough, VATSx bx = UIP-pattern with poorly-formed granulomas
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What’s not IPF?
73 yo F never-smoker, retired teacher, with chronic UTI on nitrofurantoin for > 4 years
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What’s not IPF?
53 yo F, executive secretary, uses her indoor hot tub daily, now with 4 months increasing dyspnea
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What’s the Diagnosis?
62 yo M former smoker, retired Air Force fighter pilot, no CTD symptoms, no exposures, VATSx bx with
UIP-pattern histologyProp
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Therapeutic Maneuvers
O2 – keep SpO2 > 89% regardless of activity level Improves fatigue, functioning, dyspnea
Pulmonary rehabilitation Improves fatigue, functioning, QOL
VaccinationsProp
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Therapeutic Maneuvers
Probably look for and treat GERD, OSA, PH
Education – knowledge is power
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Therapeutic Maneuvers
Anti-fibrotics
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Pathogenesis of IPF
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Anti-fibrotics work here
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Nintedanib or Pirfenidone
PlaceboPlacebo
0
‐100
‐200Placebo
Po
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King TE, et al. N Engl J Med. 2014
Pirfenidone: adverse events
Adverse Event Pirfenidone (%) (N = 278)
Placebo (%) (N = 277)
∆ (%)
Nausea 36 13.4 22.6Rash 28.1 8.7 19.4Dyspepsia 17.6 6.1 11.5Anorexia 15.8 6.5 9.3GERD 11.9 6.5 5.4Weight Loss 12.6 7.9 4.7Insomnia 11.2 6.5 4.7Dizziness 17.6 13 4.6Vomiting 12.9 8.7 4.2
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Richeldi L, et al. N Engl J Med. 2014;370(22):2071‐2082.
Nintedanib: Adverse Events
Event
INPULSIS-1 INPULSIS-2
Nintedanib (n = 309)
Placebo (n = 204)
Nintedanib (n = 329)
Placebo (n = 219)
Any (%) 96 89 94 90
Diarrhea (%) 62 19 63 18
Nausea (%) 23 6 26 7
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Summary – Main Points
IPF = idiopathic + UIP
Thorough history, exam and HRCT
Therapeutic approach: anti-fibrotics
Simple things most important: Disease education O2 Pulmonary rehab
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