SLE Flashcards
defile SLE
chronic, multisystem infalmmatory disorder of autoimmune aetiology with the potential for a fatal outcome
SLE occurs predominantly in
young women
SLE pathogenesis
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
autoantibodies present
years before first symptom
X chromosome factors
X chromosome carries predisposing genes
IRAK1, MECP2, TLR7
do men with klinefelters syndrome get SLE
14 fold increase in SLE frequency due to additional X chromosome (XXY)
do women with turners get SLE
underrepresented in women with SLE due to loss of X chromosome (X0)
why female predisposition
- oestrogen exposure likely plays a role
- presence of predisposing genes on X chromosome
environmental risk factors for SLE
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
SLE first presents with one of several of
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
lab support for SLE
complete blood count (cytopaenias)
comprehensive metabolic profile (TSH, creatinine)
creatinekinase
ESR and/or CRP
urinalysis (stick + microscopy + protein/creatinin ratio)
gold standard for diagnosis of SLE
no gold standard
lack of accurate biomarkers
wide range of clinical symptoms with inter patient variation
overlap with other connective tissue diseases
pre pregnancy evaluation in patients with SLE
all women should be advised to plan pregnancy
few absolute contraindications (active disease)
during pregnancy of patients with SLE
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)
post partum for patients with SLE
monitoring every 4-6 weeks (20% flare rate) for 3-6 months
neonatal lupus
passively acquired auto immune disease due to transplacental passage of maternal IgG anto0SSA/Ro or anti-SSB/La antibodies
occurs in 2-5% pregnancies
neonatal lupus consists of
haematological abnormalities (cytopaenias)
skin disease (rash)
heart block (1-3 degree) mainly in weeks 16-24
progression of neonatal lupus
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
general treatment points for SLE
sun protection
fish oil supplements
hyperlipidaemia
for patients on glucocorticoids: osteoporosis prophylaxis
exercise
smoking cessation as smokers have more active disease
avoid live vaccines
SLE specific treatment
topical steroids and tacrolimus
NSAIDs
antimalarials (mostly hydroxychloroquine)
glucocorticoids
immunosuppressive agents
antimalarials for use in SLE
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
biologics for treatment of SLE
- B cell depletion (anti-CD20)
- T cell costimulation inhibition
- anti-cytokine
progression of SLE
eventual outcome of death
large variation in mortality based on gender, age, ethnicity, health care system and disease progression
causes of death in SLE
infectious complication in immunosuppressant drug use
cardiovascular event ( vessel wall inflammation and/or hyper coagulability)
disease flares
ability to attain remission in SLE
remission in uncommon, and even when achieved it is rarely sustained
