ILD Flashcards
List 4 radiological features of usual interstitial pneumonia (UIP)
Subpleural reticulation
Apical-basal gradient
Honey combing
Tractional bronchiectasis
If all present - enough to make diagnosis of UIP
List 4 radiological features of non-specific interstitial pneumonia (NSIP)
Subpleural sparing
Apical-basal gradient
Ground glass change
Traction bronchiectasis
What radiological feature differentiates between UIP and NSIP?
NSIP - subpleural sparing
UIP - subpleural reticulation
Explain the relationship between UIP and IPF
Idiopathic pulmonary fibrosis (IPF) is characterised by UIP pattern
But UIP pattern is not exclusive to IPF
How is IPF diagnosed?
Radiological features of UIP
Exclusion of other known causes of pulmonary fibrosis (e.g. CTD, drugs, occupational/environmental exposures)
Diagnosis at MDM is mandatory
Surgical biopsy is rarely required
Risk factors for IPF
Older age (typically 6-7th decade) Male Ex-smoker nDust/pollution exposure GORD FHx of ILD Absence of an alternative cause for ILD
What must occur prior to lung biopsy for ILD?
MDM
When might you do a lung biopsy for ILD?
Chest HRCT pattern shows probable UIP but indeterminate
Need MDM first
Treatment for IPF
Pirfenidone
Nintedanib
Both drugs slow rate of disease progression (slows decline in FVC) and improves survival
MOA for pirfenidone
Acts through TGF-B and reduces fibroblast proliferation
MOA for nintedanib
Inhibits multiple tyrosine kinases (PDGF, VEGF, FGF)
AE pirfenidone
Nausea
Photosensitivity rash
AE nintedanib
Diarrhoea
Weight loss
Possible CV risk - avoid in those with high CV risk
Possible increase in bleeding risk - avoid in those on anticoagulants/antiplatelets
Which genes are implicated in pulmonary fibrosis?
TERT
TERC
RTEL1
PARN
What genetic syndrome can be associated with pulmonary fibrosis?
Telomeropathy syndrome = short telomeres
Associated with genetic anticipation + evolving genetic phenotype
Onset of disease becomes younger in successful generations
Can get development of other disease e.g. haematological cancers in successful generations
ILD is diffuse parenchymal ILD and can be categorised into 7 main groups. What are they?
1) Idiopathic interstitial pneumonias
2) Iatrogenic/drug induced e.g. MTX, amiodarone, bleomycin, nitrofurantoin, RT
3) Occupation/environmental e.g. asbestos, silicosis, pneumoconiosis, farmer’s lung, hypersensitivity pneumonitis
4) Granulomatous e.g. sarcoidosis
5) Collagen-vascular disease e.g. SLE, RA, scleroderma, Sjogren’s, dermatomyositis
6) Inherited e.g. alpha-1-AT deficiency
7) Unique entities e.g. LAM
Alpha-1-AT deficiency causes 3 lung conditions
ILD
Emphysema
Bronchiectasis
Idiopathic interstitial pneumonia
Major types
Rare types
Unclassified types
No known cause!
Major types
- IPF
- NSIP
- Respiratory bronchiolitis-ILD (smoking related)
- Desquamative interstitial pneumonia (smoking related)
- Cryptogenic organising pneumonia
- Acute interstitial pneumonia
Rare types
Unclassified types
- If they don’t fit into any of the other groups
Examination ILD
+/- clubbing
Fine inspiratory creps
Exertional dyspnoea - 6MWT
+/- Signs of associated disease e.g. scleroderma
+/- Signs of pulmonary HTN, cor pulmonale
Investigations ILD
RFTs
Reduced DLCO (affected first)
Reduced lung volume
6MWT Exertional hypoxemia (classic in ILD) - sats <88%
CXR
HRCT
- Prone and supine films
- Inspiratory and expiratory films
CT panel - RF, CCF, ANA, ENA, ANCA< ESR, CRP, ACE, myositis panel
CXR ILD
Early disease CXR may be normal
Late disease Bilateral Diffuse infiltrate - In IPF, get more peripheral, lower lobe > upper lobe changes Small lung volumes
Why do supine and prone HRCT?
Early IPF: Predominantly lower lobe disease and subpleural
- In higher BMI patients, often get atelectasis in the lower bases and subpleurally when lying on their backs (supine) which can look like early stage disease
- When you flip them over and prone them and the changes go away, then it was just atelectasis and gravity, if the changes stay, then its early ILD
Who needs a biopsy for ILD?
When the scan doesn’t match the clinical picture
OR
Diagnosis is needed to change management
Treatment for sarcoidosis induced ILD
Prednisolone
If resistant or breakthrough, MTX
Treatment for CT induced ILD
Prednisolone
Mycophenolate
For most
EXCEPT sarcoidosis - then its prednisolone and MTX
EXCEPT scleroderma ILD - then don’t give prednisolone (can precipitate scleroderma renal crisis)
Treatment for inflammatory ILD
High dose steroids for months
Bactrim
PPI
With one exception - scleroderma ILD - don’t give steroids
Treatment for fibrotic ILD
Treat the fibrosis
Treatment of IPF
2 drugs approved only in Australia
Slows progression of disease
Only curative option is lung transplant
Pirfenidone
- Anti-fibrotic
- Slows FVC decline by 50%
- AE: photosensitivity, GI upset
Nintedanib
- TKi
- Slows FVC decline by 50%
- AE: diarrhoea
No head to head trial
DLCO need to be >30%, FVC >50% pred = if end stage disease then not eligible
What is required for prescription of new medications for IPF?
MDT
