SLE

Question 1

What is the prevelance of SLE?

Answer 1

1 in 2000

Question 2

Which population is SLE more common in?

Answer 2

Black females

Question 3

What is the most common system affected by SLE?

Answer 3

Joints > skin > pleuropericardium > kidney > mucous membranes > CNS

Question 4

What is the standard background Rx?

Answer 4

Hydroxychloroquine

Question 5

What are some of the SE of MMF?

Answer 5

Leucopenia and diarrhoea

Question 6

If a woman has a pregnancy with foetal heart block and SS-A positive, what is the risk of foetal heart block in subsequent pregnancies?

Answer 6

25%
May be varying degrees of heart block
Needs neonatal cardiologist present to insert neonatal pacemaker

Question 7

What are the disease severity markers in SLE?

Answer 7

C3, C4, dsDNA Ab, CRP, ESR, proteinuria

Question 8

How is severe organ-threatening SLE treated?

Answer 8

Methylpred, cyclophosphamide (CTX).

MMF may be used in place of CTX.

Question 9

What are the stages of lupus nephritis?

Answer 9

I - minimal mesangial
II - mesangial proliferative GN
III - focal proliferative GN
IV - diffuse proliferative (most common) GN
V - membranous GN
VI - advanced sclerosing GN

Question 10

Which complement stain on direct immunofluorescence is pathognomonic in stage IV lupus nephritis?

Answer 10

C1q - highly specific

IgM, IgA, IgG , C3 are also usually present - termed “full house”

Question 11

What is used for maintenance immunosuppression in lupus nephritis?

Answer 11

Azathioprine or mycophenolate

Question 12

What tests should be ordered for antiphospholipid syndrome?

Answer 12

Lupus anticoagulant, Anti-cardiolipin Ab, beta2-glycoprotein 1 Ab

Question 13

How does belimumab work?

Answer 13

By blocking BLys (BAFF) from binding to BR3 on B cells

- Leads to apoptosis and inhibition of B cell maturation

Question 14

What does does electron microscopy show in Grade IV lupus nephritis?

Answer 14

Subendothelial immune complex deposits