SLE Flashcards
what are the 3 “related diseases” to systemic lupus erythematous?
Polymyositis/Dermatomyositis
Sjögren’s syndrome
Sarcoidosis
what is systemic lupus erythematous (SLE)?
Inflammatory autoimmune disease affecting many organs (esp. kidneys), with remission and exacerbations, varying from mild to life threatening
(Broad spectrum of illness)
who is the typical pt with SLE?
Mostly young women of reproductive age.
African Americans
pathophys of SLE?
B cell hyperactivity, production of pathogenic autoantibodies
what must be ruled out when a person presents like SLE?
Consider infection… especially with constitutional symptoms and if on immunosuppressants
two skin manifestations of SLE?
Butterfly rash— called malar (across the cheeks) and is classic
Alopecia (non-scarring : aka hair will grow back
joint manifestations of SLE?
- Arthritis/arthralgias-pain and/or inflammation (nondeforming - tendon laxity)
- Tendon subluxation- partial dislocation- (called Jaccoud’s). “Reducible”- can re-algne,
what may you see on Xray: SLE?
Vanishing/Shrinking Lung:
Dyspnea, restrictive PFT pattern, elevating hemidiaphragm
neurologic manifestations: SLE ? (3)
neuropathy
cognitive dysfunction
mood disorder/pyschosis (VERY COMMON)
blood manifestations: SLE?
potential for a hypercoag state
- Antiphospholipid antibody syndrome AKA anticardiolipin antibody (ACLA)=Antibodies to phospholipids increase clotting
- False positive RPR (syphillis test- false positive)
why is SLE + pregnancy so bad?
hypercoag state- miscarriage, fetal anomoly and danger to mother
bone marrow manifestations: SLE
anemia, thrombocytopenia, etc
Anything cell line can be decreased
labs for SLE
CBC: any cell line decreased
UA: protein, casts
low complement proteins (C3, C4, CH50)- immune system
serology tests for what?
test for antibodies
specific test for immune diseases
serology for SLE + related?
- ANA positive (MUST have)
- anti-phospholipid antibodies
- DS DNA (if positive = SLE)
inflammation markers:
- ESR/CRP: high
- compliment: low
DS DNA (double-stranded DNA antibody)- is it helpful if its negative or positive?
only if its positive- confirms SLE
- strong association with kidney disease
what is ANA?
anti-nuclear antibody (positive for SLE)
kidney manifestation: SLE ?
glomerulonephritis
SLE txt guidlines:
tailor treatment to ..?
If end organ involved, then …?
prevention of minor or major flares?
- tailor treatment to level of severity
- If end organ involved, then more aggressive treatment
- can only prevent minor flares (steroids)
why do SLE pts need to avoid the sun?
UVB increases flares
meds for SLE: symptomatic joint/ other pain
NSAIDs + analgesics (narcotics, acetaminophen)
meds for SLE: immunomodulating
- hydrochloroquine (DMARD)
- vit D (for nephropathy)
mainstay of txt for SLE flares? what are the 3 varying doses?
STEROIDS! WORK FAST! Prednisone: Low dose <10mg and used for mild flares Moderate dose <20mg High dose >20mg, usually 40-60mg For severe symptoms/ Major organ involvement
how long does it take steroids to have full effect? start high or low?
- starts in 24-48 hours, full effect seen in days to weeks
- Start high and then slowly taper looking for emergence of symptoms
what are the 3 immunosuppressant (steroid sparing) drugs for SLE?
Cyclophosphamide—most powerful
Azathioprine
Methotrexate—work best for joints
how do you prescribe steroids and immunosuppressants together for SLE?
keep on steroids, add immunosuppressant, as they get better- taper down steroids
txt for thrombotic events caused by SLE
fractionated heparin or warfarin
used only if they have anti-phospholipid syndrome as well
3 causes of death from SLE
- Infection: immunosuppression mostly from meds, occasionally from SLE
- Lupus flare
- Accelerated atherosclerosis/thrombosis
