SLE Flashcards

1
Q

what are the 3 “related diseases” to systemic lupus erythematous?

A

Polymyositis/Dermatomyositis
Sjögren’s syndrome
Sarcoidosis

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2
Q

what is systemic lupus erythematous (SLE)?

A

Inflammatory autoimmune disease affecting many organs (esp. kidneys), with remission and exacerbations, varying from mild to life threatening
(Broad spectrum of illness)

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3
Q

who is the typical pt with SLE?

A

Mostly young women of reproductive age.

African Americans

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4
Q

pathophys of SLE?

A

B cell hyperactivity, production of pathogenic autoantibodies

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5
Q

what must be ruled out when a person presents like SLE?

A

Consider infection… especially with constitutional symptoms and if on immunosuppressants

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6
Q

two skin manifestations of SLE?

A

Butterfly rash— called malar (across the cheeks) and is classic
Alopecia (non-scarring : aka hair will grow back

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7
Q

joint manifestations of SLE?

A
  1. Arthritis/arthralgias-pain and/or inflammation (nondeforming - tendon laxity)
  2. Tendon subluxation- partial dislocation- (called Jaccoud’s). “Reducible”- can re-algne,
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8
Q

what may you see on Xray: SLE?

A

Vanishing/Shrinking Lung:

Dyspnea, restrictive PFT pattern, elevating hemidiaphragm

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9
Q

neurologic manifestations: SLE ? (3)

A

neuropathy
cognitive dysfunction
mood disorder/pyschosis (VERY COMMON)

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10
Q

blood manifestations: SLE?

A

potential for a hypercoag state

  • Antiphospholipid antibody syndrome AKA anticardiolipin antibody (ACLA)=Antibodies to phospholipids increase clotting
  • False positive RPR (syphillis test- false positive)
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11
Q

why is SLE + pregnancy so bad?

A

hypercoag state- miscarriage, fetal anomoly and danger to mother

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12
Q

bone marrow manifestations: SLE

A

anemia, thrombocytopenia, etc

Anything cell line can be decreased

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13
Q

labs for SLE

A

CBC: any cell line decreased
UA: protein, casts
low complement proteins (C3, C4, CH50)- immune system

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14
Q

serology tests for what?

A

test for antibodies

specific test for immune diseases

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15
Q

serology for SLE + related?

A
  • ANA positive (MUST have)
  • anti-phospholipid antibodies
  • DS DNA (if positive = SLE)

inflammation markers:

  • ESR/CRP: high
  • compliment: low
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16
Q

DS DNA (double-stranded DNA antibody)- is it helpful if its negative or positive?

A

only if its positive- confirms SLE

- strong association with kidney disease

17
Q

what is ANA?

A

anti-nuclear antibody (positive for SLE)

18
Q

kidney manifestation: SLE ?

A

glomerulonephritis

19
Q

SLE txt guidlines:
tailor treatment to ..?
If end organ involved, then …?
prevention of minor or major flares?

A
  • tailor treatment to level of severity
  • If end organ involved, then more aggressive treatment
  • can only prevent minor flares (steroids)
20
Q

why do SLE pts need to avoid the sun?

A

UVB increases flares

21
Q

meds for SLE: symptomatic joint/ other pain

A

NSAIDs + analgesics (narcotics, acetaminophen)

22
Q

meds for SLE: immunomodulating

A
  • hydrochloroquine (DMARD)

- vit D (for nephropathy)

23
Q

mainstay of txt for SLE flares? what are the 3 varying doses?

A
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
24
Q

how long does it take steroids to have full effect? start high or low?

A
  • starts in 24-48 hours, full effect seen in days to weeks

- Start high and then slowly taper looking for emergence of symptoms

25
what are the 3 immunosuppressant (steroid sparing) drugs for SLE?
Cyclophosphamide—most powerful Azathioprine Methotrexate—work best for joints
26
how do you prescribe steroids and immunosuppressants together for SLE?
keep on steroids, add immunosuppressant, as they get better- taper down steroids
27
txt for thrombotic events caused by SLE
fractionated heparin or warfarin | used only if they have anti-phospholipid syndrome as well
28
3 causes of death from SLE
1. Infection: immunosuppression mostly from meds, occasionally from SLE 2. Lupus flare 3. Accelerated atherosclerosis/thrombosis