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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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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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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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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 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 examination
Hands Chest
Crackles ANY VELCRO CRACKLE IS ABNORMAL MAKE SURE YOU LISTEN AT THE BASES
Joints Skin Prop
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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
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
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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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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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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