ILD

Question 1

List 4 radiological features of usual interstitial pneumonia (UIP)

Answer 1

Subpleural reticulation
Apical-basal gradient
Honey combing
Tractional bronchiectasis

If all present - enough to make diagnosis of UIP

Question 2

List 4 radiological features of non-specific interstitial pneumonia (NSIP)

Answer 2

Subpleural sparing
Apical-basal gradient
Ground glass change
Traction bronchiectasis

Question 3

What radiological feature differentiates between UIP and NSIP?

Answer 3

NSIP - subpleural sparing

UIP - subpleural reticulation

Question 4

Explain the relationship between UIP and IPF

Answer 4

Idiopathic pulmonary fibrosis (IPF) is characterised by UIP pattern

But UIP pattern is not exclusive to IPF

Question 5

How is IPF diagnosed?

Answer 5

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

Question 6

Risk factors for IPF

Answer 6

Older age (typically 6-7th decade)
Male
Ex-smoker
 nDust/pollution exposure
GORD
FHx of ILD
Absence of an alternative cause for ILD

Question 7

What must occur prior to lung biopsy for ILD?

Answer 7

MDM

Question 8

When might you do a lung biopsy for ILD?

Answer 8

Chest HRCT pattern shows probable UIP but indeterminate

Need MDM first

Question 9

Treatment for IPF

Answer 9

Pirfenidone
Nintedanib

Both drugs slow rate of disease progression (slows decline in FVC) and improves survival

Question 10

MOA for pirfenidone

Answer 10

Acts through TGF-B and reduces fibroblast proliferation

Question 11

MOA for nintedanib

Answer 11

Inhibits multiple tyrosine kinases (PDGF, VEGF, FGF)

Question 12

AE pirfenidone

Answer 12

Nausea

Photosensitivity rash

Question 13

AE nintedanib

Answer 13

Diarrhoea
Weight loss
Possible CV risk - avoid in those with high CV risk
Possible increase in bleeding risk - avoid in those on anticoagulants/antiplatelets

Question 14

Which genes are implicated in pulmonary fibrosis?

Answer 14

TERT
TERC
RTEL1
PARN

Question 15

What genetic syndrome can be associated with pulmonary fibrosis?

Answer 15

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

Question 16

ILD is diffuse parenchymal ILD and can be categorised into 7 main groups. What are they?

Answer 16

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

Question 17

Alpha-1-AT deficiency causes 3 lung conditions

Answer 17

ILD
Emphysema
Bronchiectasis

Question 18

Idiopathic interstitial pneumonia
Major types
Rare types
Unclassified types

Answer 18

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

Question 19

Examination ILD

Answer 19

+/- clubbing
Fine inspiratory creps
Exertional dyspnoea - 6MWT
+/- Signs of associated disease e.g. scleroderma
+/- Signs of pulmonary HTN, cor pulmonale

Question 20

Investigations ILD

Answer 20

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

Question 21

CXR ILD

Answer 21

Early disease CXR may be normal

Late disease
Bilateral 
Diffuse infiltrate
- In IPF, get more peripheral, lower lobe > upper lobe changes
Small lung volumes

Question 22

Why do supine and prone HRCT?

Answer 22

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

Question 23

Who needs a biopsy for ILD?

Answer 23

When the scan doesn’t match the clinical picture
OR
Diagnosis is needed to change management

Question 24

Treatment for sarcoidosis induced ILD

Answer 24

Prednisolone

If resistant or breakthrough, MTX

Question 25

Treatment for CT induced ILD

Answer 25

Prednisolone
Mycophenolate
For most

EXCEPT sarcoidosis - then its prednisolone and MTX
EXCEPT scleroderma ILD - then don’t give prednisolone (can precipitate scleroderma renal crisis)

Question 26

Treatment for inflammatory ILD

Answer 26

High dose steroids for months
Bactrim
PPI

With one exception - scleroderma ILD - don’t give steroids

Question 27

Treatment for fibrotic ILD

Answer 27

Treat the fibrosis

Question 28

Treatment of IPF

Answer 28

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

Question 29

What is required for prescription of new medications for IPF?

Answer 29

MDT