Multisystem Disorders

Question 1

Which autoimmune antibodies are tested in granulomatosis with polyangiitis?

Answer 1

cANCA - in 90%

pANCA - in 25% (more associated with Churg-straus)

Question 2

What kidney disease do people with granulomatosis with polyangiitis get?

Answer 2

Rapidly progressive glomerulonephritis, that leads to Chonric kidney failure.

Dx: renal biopsy with crescent shaped scars in the glomeruli.

When tested for immunofluorence for IgG it is negative or ‘pauci-immune’ in 80% cases
- this feature differentiates it from goodpastures syndrome.

Question 3

Respiratory differences between Chrug Strauss and GPA?

Answer 3

CS - dyspnoea, asthma (eosinphillia)

GPA - dyspnoea, haemoptysis, epistaxis (saddle shaped nose)

Question 4

What causes the hypercalcaemia in sarcoidosis?

Answer 4

Macrophages in the granulomas cause increased conversion of vit D to its active form (1,25- dihydroxycholecalciferol)

Question 5

What are the 3 stages of Churg strass disease ?

Answer 5

Allergy - asthma, nasal polyps

Eosinophilia

Vasculitis (kidneys and petechia rash)

Question 6

What skin condition is pathognomonic of sarcoidosis

Answer 6

Lupus pernio (purple raised lesions on face)

Question 7

Raised calcium and erythema nodosum and lung disease suggest what diagnosis?

Answer 7

Sarcoidosis

Question 8

What initial test is most helpful to test pulmonary status in someone with a sarcoidosis exacerbation?

Answer 8

PulmonRy function tests with Transfer factor

TF is sensitive

Question 9

How does diffuse alveolar haemorrhage present and what’s it associated with?

Answer 9

Associated with SLE (can be fatal)
Sudden onset dyspnoea with or without haemoptysis, CXR with diffuse infiltrates, in pulmonary haemorrhage there is a high DLCO (diffusing captaincy for carbon monoxide)