SLE Flashcards

1
Q

defile SLE

A

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

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2
Q

SLE occurs predominantly in

A

young women

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3
Q

SLE pathogenesis

A

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

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4
Q

autoantibodies present

A

years before first symptom

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5
Q

X chromosome factors

A

X chromosome carries predisposing genes
IRAK1, MECP2, TLR7

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6
Q

do men with klinefelters syndrome get SLE

A

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

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7
Q

do women with turners get SLE

A

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

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8
Q

why female predisposition

A
  1. oestrogen exposure likely plays a role
  2. presence of predisposing genes on X chromosome
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9
Q

environmental risk factors for SLE

A

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

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10
Q

SLE first presents with one of several of

A

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

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11
Q

lab support for SLE

A

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

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12
Q

gold standard for diagnosis of SLE

A

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

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13
Q

pre pregnancy evaluation in patients with SLE

A

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

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14
Q

during pregnancy of patients with SLE

A

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)

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15
Q

post partum for patients with SLE

A

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

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16
Q

neonatal lupus

A

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

17
Q

neonatal lupus consists of

A

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

18
Q

progression of neonatal lupus

A

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

19
Q

general treatment points for SLE

A

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

20
Q

SLE specific treatment

A

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

21
Q

antimalarials for use in SLE

A

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

22
Q

biologics for treatment of SLE

A
  1. B cell depletion (anti-CD20)
  2. T cell costimulation inhibition
  3. anti-cytokine
23
Q

progression of SLE

A

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

24
Q

causes of death in SLE

A

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

25
Q

ability to attain remission in SLE

A

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