ILD

Question 1

Sarcoidosis staging

Answer 1

Stage I - hilar lymphadenopathy (50%)
Stage II - hilar lymphadenopathy + pulmonary infiltrates (25%)
Stage III - pulmonary infiltrates (15%)
Stage IV - pulmonary fibrosis (10%)

Question 2

Lofgrens syndrome

Answer 2

Fever
bilateral hilar lymphadenopathy
erythema nodosum
arthralgia
sarcoid

80% spontaneous remission in 12 years

Question 3

Heerfordt’s syndrome

Answer 3

sarcoidosis
facial nerve palsy
parotitis
anterior uveitis

Question 4

Causes of granulomatous disease

Answer 4

sarcoid
TB/NTM
HP
GPA
Drugs - Ab/Ib/Ept
fungal
beryliosis
RA
Malignancy
IBD

Question 5

Markers of poor prognosis in sarcoidosis

Answer 5

lupus pernio
nasal mucosa involvement
chronic uveitis
chronic hypercalcaemia
nephrocalcinosis
neural involvement
>40 years old
black race

Question 6

Nintedanib - actions and dose

Answer 6

tyrosine kinase inhibitor
150mg BD

reduces time to exacerbation

Question 7

nintedanib - Contraindications

Answer 7

anticoagulation - increases risk of bleeding
peanut/soy allergy
liver disease
pregnancy + breast feeding

Question 8

nintedanib - SE

Answer 8

diarrhoea, nausea, vomiting, weight loss, abnormal LFTs

Question 9

Pirfenidone - actions and dose

Answer 9

inhibits collagen synthesis and reduces fibroblast proliferation
267mg TDS

Question 10

pirfenidone - CI

Answer 10

pregnancy and breast feeding
smoking
eGFR <30
child pugh C liver disease
<50% FVC >80%