systemic lupus erythematous (SLE)

Question 1

what is it?

Answer 1

systemic autoimmune condition where inflammation in organs causes acute illness

Question 2

what does it involve?

Answer 2

skin, joints, kidneys, blood cells and NS

Question 3

what are autoimmune causes?

Answer 3

genetic predisposition - HLA genes
hormonal factors - inc. oestrogen
environmental factors - UV light, bacterial/viral infection, some meds

Question 4

what are risk factors?

Answer 4

women - 20-40
smokers
Afro-Caribbean, Hispanic, American, Asian and Chinese

Question 5

what type of hypersensitivity reaction is it?

Answer 5

type 3

Question 6

what happens in type 3 hypersensitivity reactions?

Answer 6

immune system attacks cells and tissues resulting in inflammation and tissue damage, involves formation of immune complexes

Question 7

what is the pathophysiology?

Answer 7

1. loss of immune regulation = inc. defective apoptosis
2. necrotic cells release nuclear materials act as auto-antigens
3. auto-immunity results from exposure to nuclear and cellular auto-antigens
4. B and T cells stimulated and autoantibodies produced
5. auto-antigens and autoantibodies form immune complexes that circulate and become deposited n basement membrane (type 3 HS)
6. activation of complement which attracts leukocytes which release cytokines
7. cytokine release perpetuates inflammation which causes necrosis and scarring

Question 8

what is clinical presentation of mild, moderate and severe?

Answer 8

mild = simple cutaneous changes
moderate = arthritis, effusions
severe = organ-threatening disease

Question 9

what are non-specific symptoms?

Answer 9

fever
fatigue
weight loss

Question 10

what are cutaneous features?

Answer 10

photosensitive rash
non-scarring alopecia
oral/nasal ulcers
raynauds

Question 11

what is renal systemic involvement?

Answer 11

lupus nephritis
proteinuria >0.5 in 24 hours

Question 12

what is neurological involvement?

Answer 12

seizures, psychosis psychiatric change (delirium psychosis), headache, cranial nerve disorder

Question 13

what is serositis involvement?

Answer 13

pleuritis or pericarditis

Question 14

what is haematological involvement (common)?

Answer 14

leukopenia
lymphadenopathy
thrombocytopenia
haemolytic anaemia

Question 15

what investigations are used?

Answer 15

autoantibodies

Question 16

which autoantibody is most specific?

Answer 16

anti-dsDNA

Question 17

what antibody is seen in drug induced lupus?

Answer 17

anti-histone antibodies

Question 18

what is 1st line management?

Answer 18

sun protection
minimize steroid use

Question 19

what is 2nd line management?

Answer 19

hydroxychloroquine + NSAIDs

Question 20

when are NSAIDs contraindicated?

Answer 20

lupus nephritis

Question 21

what is 3rd line management?

Answer 21

hydroxychloroquine