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
Q

what are the 3 immunosuppressant (steroid sparing) drugs for SLE?

A

Cyclophosphamide—most powerful
Azathioprine
Methotrexate—work best for joints

26
Q

how do you prescribe steroids and immunosuppressants together for SLE?

A

keep on steroids, add immunosuppressant, as they get better- taper down steroids

27
Q

txt for thrombotic events caused by SLE

A

fractionated heparin or warfarin

used only if they have anti-phospholipid syndrome as well

28
Q

3 causes of death from SLE

A
  1. Infection: immunosuppression mostly from meds, occasionally from SLE
  2. Lupus flare
  3. Accelerated atherosclerosis/thrombosis