SLE

Question 1

defile SLE

Answer 1

chronic, multisystem infalmmatory disorder of autoimmune aetiology with the potential for a fatal outcome

Question 2

SLE occurs predominantly in

Answer 2

young women

Question 3

SLE pathogenesis

Answer 3

genetic predisposition
immune abnormalities due to a loss of self tolerance
autoantibodies form immune complexes with self antigens
characteristics of immune complexes define inflammatory properties

Question 4

autoantibodies present

Answer 4

years before first symptom

Question 5

X chromosome factors

Answer 5

X chromosome carries predisposing genes
IRAK1, MECP2, TLR7

Question 6

do men with klinefelters syndrome get SLE

Answer 6

14 fold increase in SLE frequency due to additional X chromosome (XXY)

Question 7

do women with turners get SLE

Answer 7

underrepresented in women with SLE due to loss of X chromosome (X0)

Question 8

why female predisposition

Answer 8

1. oestrogen exposure likely plays a role
2. presence of predisposing genes on X chromosome

Question 9

environmental risk factors for SLE

Answer 9

infections: EBV, flares after bacterial infections
ultraviolet light
silica dust
allergies to medications, particularly antibiotics
higher prevalence of lupus in pet dogs of SLE patients

Question 10

SLE first presents with one of several of

Answer 10

unexplained non specific symptoms such as fever, fatigue, weight loss
photosensitive rash
arthralgia or arthritis
Raynaud phenomenon
serositis
nephritis/nephrotic syndrome
neurological symptoms including seizures or psychosis
alopecia
phlebitis
recurrent miscarriage
anaemia

Question 11

lab support for SLE

Answer 11

complete blood count (cytopaenias)
comprehensive metabolic profile (TSH, creatinine)
creatinekinase
ESR and/or CRP
urinalysis (stick + microscopy + protein/creatinin ratio)

Question 12

gold standard for diagnosis of SLE

Answer 12

no gold standard
lack of accurate biomarkers
wide range of clinical symptoms with inter patient variation
overlap with other connective tissue diseases

Question 13

pre pregnancy evaluation in patients with SLE

Answer 13

all women should be advised to plan pregnancy
few absolute contraindications (active disease)

Question 14

during pregnancy of patients with SLE

Answer 14

regular review of:
- effect of pregnancy on diseases (disease flares in 60% of pregnancies, mostly minor, managed with symptomatic therapy or low CS)
- effect of disease on pregnancy (CHB, growth retardation, placental flow)

Question 15

post partum for patients with SLE

Answer 15

monitoring every 4-6 weeks (20% flare rate) for 3-6 months

Question 16

neonatal lupus

Answer 16

passively acquired auto immune disease due to transplacental passage of maternal IgG anto0SSA/Ro or anti-SSB/La antibodies
occurs in 2-5% pregnancies

Question 17

neonatal lupus consists of

Answer 17

haematological abnormalities (cytopaenias)
skin disease (rash)
heart block (1-3 degree) mainly in weeks 16-24

Question 18

progression of neonatal lupus

Answer 18

disappears with the clearance of maternal antibodies by the 6th to 8th month of postnatal life (except for congenital heart block)
congenital heart block carries 15-20% mortality risk

Question 19

general treatment points for SLE

Answer 19

sun protection
fish oil supplements
hyperlipidaemia
for patients on glucocorticoids: osteoporosis prophylaxis
exercise
smoking cessation as smokers have more active disease
avoid live vaccines

Question 20

SLE specific treatment

Answer 20

topical steroids and tacrolimus
NSAIDs
antimalarials (mostly hydroxychloroquine)
glucocorticoids
immunosuppressive agents

Question 21

antimalarials for use in SLE

Answer 21

mostly hydroxychloroquine
400-800mg daily until remission, maintenance with 200-400mg
treats:
- most types of skin disease
- arthritis/arthralgia/severe fatigue
for all patients:
- reduces risk of flare
reduce damage to kidneys and CNS
- has antithrombotic and antihyperlipidaemic effects

Question 22

biologics for treatment of SLE

Answer 22

1. B cell depletion (anti-CD20)
2. T cell costimulation inhibition
3. anti-cytokine

Question 23

progression of SLE

Answer 23

eventual outcome of death
large variation in mortality based on gender, age, ethnicity, health care system and disease progression

Question 24

causes of death in SLE

Answer 24

infectious complication in immunosuppressant drug use
cardiovascular event ( vessel wall inflammation and/or hyper coagulability)
disease flares

Question 25

ability to attain remission in SLE

Answer 25

remission in uncommon, and even when achieved it is rarely sustained