interstitial lung disease - rcpmedicine.co.uk · 16.01.2019 · interstitial lung disease dr. ian...
TRANSCRIPT
Interstitial Lung Disease
Dr. Ian Forrest
Consultant Respiratory Physician
Clinical Lead NILDS
Royal Victoria Infirmary
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
Interstitium
Fibrosis
A
B
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
ILDs>200
CTD - ILD
ChronicHypersensitivity
Pneumonitis
Idiopathic Pulmonary
Fibrosis
Idiopathic Pulmonary Fibrosis (IPF)
One in a hundred people in the UK will die of IPF
Pathogenesis of IPF
Goodwin AT, Jenkins G. CHEST 2016; 149(1):228-237
Familial ILD• 2-20% of cases in UK
• Autosomal dominant inheritance with incomplete penetrance
• Phenotypic heterogeneity in families
• 100,000 genomes study
Pathogenesis of IPF
Goodwin AT, Jenkins G. CHEST 2016; 149(1):228-237
GORD
• Common
• Often proximal and non acid
• Fundoplication helps?
• PPI helps?
• Populates lung microbiome?
Pathogenesis of IPF
Goodwin AT, Jenkins G. CHEST 2016; 149(1):228-237
Natural History of IPF
Ley et al. Am J Respir Crit Care Med 2011, 183:431–440
Early Detection?Medical students Clinical Physiologists
Crep = French Pancake ≠ Crackle
Treatment of IPF
Nothing (much of previous treatment was harmful)
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
Trials in IPF
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
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
Integrated Palliative Care
Murray SA et al. BMJ.2005; 330(7498): 1007–1011
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
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
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
Anti-fibrotic Therapies(If MDT ratified IPF and VC 50-80%)
• Pirfenidone
• Nintedanib
• Probably suppresses fibroblast proliferation via TGF- inhibition
• Side effects: GI upset, weight loss, photosensitive rash, hepatotoxicity
• C/I: renal impairment eGFR <30
Pirfenidone
Pirfenidone rash
Nintedanib
• Triple TKI
• Side effects: GI upset, diarrhoea, hepatotoxicity
• Cautions: anticoagulants, cardiac disease, surgery, liver disease
Nintedanib Diarrhoea
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
• 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
Combined Pulmonary Fibrosis and Emphysema (CPFE)
• Spirometry can be normal precluding antifibrotics
• Gas transfer very low / desaturate++
• Pulmonary hypertension common
• Poor prognosis
Lung cancer
• Increased risk in IPF (7x)
• Independent of smoking
• Treatment can trigger AE-IPF
Acute Exacerbations of IPF (AE-IPF)
AE-IPF
• 1 in 7 patients/year
• 20-40% fatality
• Treatment?
AE-IPF• Exclude PE
• Exclude heart failure
• Exclude infection
• Support with oxygen
• Steroid
• Co-trimoxazole
• Palliative care / EOLC
• Do not intubate /ventilate
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
CTD-ILD PatternsUIP NSIP COP Diffuse Alveolar
DamageLymphocyticInterstitial Pneumonia
Diffuse AlvelolarHaemorrhage
Systemic sclerosis
++ ++++ + + - -
RA ++ + - + - -
MCTD ++ +++ - - + -
SLE + + + ++ - +++
PM/DM ++ ++++ ++ + - -
Sjogren’s + + + - +++ -
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
Anti-synthetase ILD
• High index of suspicion
• Requires specific antibody testing
• Work closely with your Rheumatologist / Neurologist
Why HP not EAA?
• Type III (immune complex) and Type IV (T-lymphocyte) DTH• Affects distal airways, alveoli and interstitium
Protocolised HRCT1mm helix done prone with sharpened protocol
In both full inspiration and expirationNo contrast
Inspiratory Expiratory
Look Carefully
Look Carefully
Look Carefully
Look Carefully
Look Carefully
Listen Carefully
HP TreatmentRemove antigen exposure
Immunosuppression
Fibrotic HP…challenging
At the ILD MDT….
IPF
CTD-ILD
CHP
Possible IPF
Possible fibroticHP
Interstitial Pneumonia with autoimmune features
(IPAF)
Unclassifiable?
At the ILD MDT….
Lung Biopsy ?
Future Fibrotic ILD Treatment (after Dr Toby Maher)
Manage disease complications
Treat Underlying Cause (where known)
Anti-fibrotic therapy
If disease progression
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
Questions…..?