Interstitial Lung Disease - rcpmedicine.co.uk · 16.01.2019 · Interstitial Lung Disease Dr. Ian...
Transcript of Interstitial Lung Disease - rcpmedicine.co.uk · 16.01.2019 · Interstitial Lung Disease Dr. Ian...
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Interstitial Lung Disease
Dr. Ian Forrest
Consultant Respiratory Physician
Clinical Lead NILDS
Royal Victoria Infirmary
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ILD – The Confusing Land of the TLA?
• Take it back to basics
• Focus on
– idiopathic pulmonary fibrosis (IPF)– connective tissue disease (CTD) – chronic hypersensitivity pneumonitis (CHP)
• Discuss the challenges of clinical practice and emerging clinical science
• Questions
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Interstitium
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Fibrosis
A
B
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ILDs>200
Known Causeeg. CTD/Drugs/
Asbestos
Idiopathic Interstitial
Pneumonias
Granulomatous Disease eg. Sarcoid and Hypersensitivity
Pneumonitis
Otherseg. LAM /LCH
OthersIdiopathic Pulmonary
Fibrosis
DIP RBILD
NSIP AIPLIP COP
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ILDs>200
CTD - ILD
ChronicHypersensitivity
Pneumonitis
Idiopathic Pulmonary
Fibrosis
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Idiopathic Pulmonary Fibrosis (IPF)
One in a hundred people in the UK will die of IPF
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Pathogenesis of IPF
Goodwin AT, Jenkins G. CHEST 2016; 149(1):228-237
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Familial ILD• 2-20% of cases in UK
• Autosomal dominant inheritance with incomplete penetrance
• Phenotypic heterogeneity in families
• 100,000 genomes study
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Pathogenesis of IPF
Goodwin AT, Jenkins G. CHEST 2016; 149(1):228-237
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GORD
• Common
• Often proximal and non acid
• Fundoplication helps?
• PPI helps?
• Populates lung microbiome?
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Pathogenesis of IPF
Goodwin AT, Jenkins G. CHEST 2016; 149(1):228-237
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Natural History of IPF
Ley et al. Am J Respir Crit Care Med 2011, 183:431–440
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Early Detection?Medical students Clinical Physiologists
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Crep = French Pancake ≠ Crackle
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Treatment of IPF
Nothing (much of previous treatment was harmful)
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Steroids in IPF
Now the drugs don't work
They just make you worse
But I know I'll see your face again……
The Verve 1997
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Trials in IPF
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Treatment of IPF• Nothing (much of previous treatment was harmful)
• Best Supportive Care ie: Oxygen / ILD rehabilitation
• Palliative Care
• Transplantation
• Recruit to Clinical Trials
• Antifibrotic therapies
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Treatment of IPF• Nothing (much of previous treatment was harmful)
• Best Supportive Care ie: Oxygen / ILD rehabilitation
• Palliative Care
• Transplantation
• Recruit to Clinical Trials
• Antifibrotic therapies
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Integrated Palliative Care
Murray SA et al. BMJ.2005; 330(7498): 1007–1011
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Treatment of IPF• Nothing (much of previous treatment was harmful)
• Best Supportive Care ie:oxygen/rehabilitation
• Palliative Care
• Transplantation
• Recruit to Clinical Trials
• Antifibrotic therapies
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Treatment of IPF• Nothing (much of previous treatment was harmful)
• Best Supportive Care ie:oxygen/rehabilitation
• Palliative Care
• Transplantation
• Recruit to Clinical Trials
• Antifibrotic therapies
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Treatment of IPF• Nothing (much of previous treatment was harmful)
• Best Supportive Care ie:oxygen/rehabilitation
• Palliative Care
• Transplantation
• Recruit to Clinical Trials
• Antifibrotic therapies
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Anti-fibrotic Therapies(If MDT ratified IPF and VC 50-80%)
• Pirfenidone
• Nintedanib
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• Probably suppresses fibroblast proliferation via TGF- inhibition
• Side effects: GI upset, weight loss, photosensitive rash, hepatotoxicity
• C/I: renal impairment eGFR <30
Pirfenidone
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Pirfenidone rash
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Nintedanib
• Triple TKI
• Side effects: GI upset, diarrhoea, hepatotoxicity
• Cautions: anticoagulants, cardiac disease, surgery, liver disease
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Nintedanib Diarrhoea
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Practical Points• These drugs are not immunosuppressive and don’t need
to be stopped with infection
• If you think there is a side effect, feel free to stop the drug in short term….bad things don’t happen
• There is (should be) an ILD nurse specialist at the end of the ‘phone…please call
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• Emphysema • Lung Cancer• Pulmonary hypertension • Venous thromboembolism• Sleep-disordered breathing • Gastroesophageal reflux disease • Coronary artery disease • Depression and anxiety• Deconditioning • Frailty and Sarcopenia
Co-Morbidities
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Combined Pulmonary Fibrosis and Emphysema (CPFE)
• Spirometry can be normal precluding antifibrotics
• Gas transfer very low / desaturate++
• Pulmonary hypertension common
• Poor prognosis
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Lung cancer
• Increased risk in IPF (7x)
• Independent of smoking
• Treatment can trigger AE-IPF
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Acute Exacerbations of IPF (AE-IPF)
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AE-IPF
• 1 in 7 patients/year
• 20-40% fatality
• Treatment?
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AE-IPF• Exclude PE
• Exclude heart failure
• Exclude infection
• Support with oxygen
• Steroid
• Co-trimoxazole
• Palliative care / EOLC
• Do not intubate /ventilate
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Enomoto N et al. BMC Pulmonary Medicine 2015 15:15
Treatment of acute exacerbation of idiopathic pulmonary fibrosis with direct
hemoperfusion using a polymyxin B-immobilized fibre column improves survival
Haemoperfusion
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CTD-ILD PatternsUIP NSIP COP Diffuse Alveolar
DamageLymphocyticInterstitial Pneumonia
Diffuse AlvelolarHaemorrhage
Systemic sclerosis
++ ++++ + + - -
RA ++ + - + - -
MCTD ++ +++ - - + -
SLE + + + ++ - +++
PM/DM ++ ++++ ++ + - -
Sjogren’s + + + - +++ -
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Immunosuppression?
NSIP (Often SSc)
• Ground glass predominant
• Peripheral, subpleural
• Basal predominant
• No honeycombing
UIP (Often RA)
• Little ground glass
• Peripheral, subpleural
• Basal predominant
• Honeycombing - key feature
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Anti-synthetase ILD
• High index of suspicion
• Requires specific antibody testing
• Work closely with your Rheumatologist / Neurologist
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Why HP not EAA?
• Type III (immune complex) and Type IV (T-lymphocyte) DTH• Affects distal airways, alveoli and interstitium
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Protocolised HRCT1mm helix done prone with sharpened protocol
In both full inspiration and expirationNo contrast
Inspiratory Expiratory
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Look Carefully
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Look Carefully
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Look Carefully
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Look Carefully
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Look Carefully
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Listen Carefully
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HP TreatmentRemove antigen exposure
Immunosuppression
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Fibrotic HP…challenging
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At the ILD MDT….
IPF
CTD-ILD
CHP
Possible IPF
Possible fibroticHP
Interstitial Pneumonia with autoimmune features
(IPAF)
Unclassifiable?
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At the ILD MDT….
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Lung Biopsy ?
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Future Fibrotic ILD Treatment (after Dr Toby Maher)
Manage disease complications
Treat Underlying Cause (where known)
Anti-fibrotic therapy
If disease progression
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In Conclusion1. One in a hundred of us will die of IPF
2. Your stethoscope remains useful
3. It’s often tricky…..MDT working is vital
4. Genetics /translational science informing treatment
5. Get rid of your feather bedding
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Questions…..?