ILD - IPF

Question 1

Genetics ax with IPF

Answer 1

50% have a single nucleotide polymorphism in MUSC5B glycoprotein gene

short telomere length also ax

Question 2

Risk factors IPF

Answer 2

Smoking
Age

Question 3

Diagnostic criteria IPF
(2)

Answer 3

Exclusion other causes eg CTD, exposure

AND one of:

UIP

or

specific HRCT or histopathology patterns subjected to lung tissue sampling

Question 4

Features of Definite UIP

Answer 4

Subpleural and basal predominant

Heterogenous

Honeycombing

+- bronchiectasis

Question 5

Features of Probable UIP

Answer 5

Subpleural and basal predominant

Heterogenous

+- bronchiectasis

+- mild GGO

but no honeycombing

Question 6

Features intermediate UIP

Answer 6

Subpleural/basal predominant

Subtle reticulation

Mild GGO or distortion

features don’t suggest any specific aetiology

Question 7

Histopathology findings UIP criteria

Answer 7

1. Remodelling lung structure
2. Often honeycomb
3. Alternates with less affected areas of lung

typically subpleural and paraseptal

Question 8

Histopathology findings UIP

Answer 8

patchy interstitial infiltrate lymphocyte and plasma cells
hyperplasia type 2 pneumocytes and bronchiolar epithelium
dense collagen
scattered convex subeptihelial foci
proliferating fibroblasts and myofibroblasts

microscopic honeycombing
cystic fibrotic airspace
mucous ad inflammatory cells

smooth muscle metaplasia

Question 10

ILD

Bloods to exclude CTD

Answer 10

ANA
RF
Myositis panel
Anti-CCP

Question 11

Why do BAL in ILD

Answer 11

Differential cell count

Question 12

What’s the dosage nintedanib

Answer 12

150mg BD

Question 13

MOA nintedanib

Answer 13

TKI

Question 14

SE nintedanib

Answer 14

Diarrhoea
Bleeding

Question 15

Cautions nintedanib

Answer 15

Bleeding risk
avoid anti-coag

Question 16

MOA Perfenidone

Answer 16

Inhibition TGF-B and collagen

Question 17

Dose perfenidone

Answer 17

801mg TDS

Question 18

SE Perfenidone

Answer 18

Nausea, photosensitivity

Question 19

Cautions perfenidone

Answer 19

Inhibition CYP-1A2 eg fluoroquinines increase levels

Inducers reduced levels eg smoking PPI

Question 20

Monitoring for nintedanib and perfenidone

Answer 20

LFTs