Respiratory - PFT And ILD

Question 1

UIP radiological changes and NSIP radiological changes.

Answer 1

UIP - subpleural reticulation, apical-basal gradient (worse at based), traction bronchiectasis. Honeycombing. Usually in IPF and rheumatoid arthritis

NSIP - ground glass changes, subpleural SPARING, traction bronchiectasis, apical to basal gradient. Usually homogenous. Usually in COnnective tissue disease and can be in drug induced.

Question 2

Antifibrotics and their side effects and their MOA in IPF.

Answer 2

Pirfenidone - acts through TGF-B and reduce fibroblast proliferation. Prescribed with sunscreen due to photosensitivity rash. Also has nausea and other GIT sx

Nintedanib - inhibits multiple tyrosine kinase. Side effects include diarrhoea, weight loss, possible cvs and bleeding risk

Question 3

What is lofgren syndrome and what is the triad?

Answer 3

Lofgren syndrome is an Acute form of sarcoidosis characterised by a trial of symptoms - erythema nodosum, bilateral hilar lymphadenopathy and arthritis. This usually self resolves within 6 months

Question 4

Difference between pirfenidone and nindetanib

Answer 4

Pirfenidone
- inhibits TGF- beta
- metabolised by CYP1a2, not to be used with fluvoxamine
- causes rash, photosensitivity, GI upset (give sunscreen)
- TDS dosing
- contraindicated in child Pugh C and CKD

Nindetanib
- inhibits PDGF, FGF, VEGF
- poor healing, increases risk of clots and thrombosis,
- contraindicated in child Pugh B and C.