Lupus (exam 1) Flashcards

1
Q

SLE

A

heterogenous illnesses that have in common the development of autoimmunity to self-nucleic acids and proteins

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2
Q

spectrum of SLE

A

skin-only disease to severe systemic involvement

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3
Q

SLE is characterized as a _______________ disease

A

chronic anti inflammatory

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4
Q

who is affected most by lupus?

A

women

starts at child bearing age

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5
Q

discoid lupus erythematous (DLE)

A

scarring that can be permanently disfiguring
limited to skin on head

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6
Q

subacute cutaneous and acute cutaneous lupus

A

highly photosensitive
nonscarring

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7
Q

acute cutaneous lupus erythematosus

A

malar rash
underlying visceral involvement

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8
Q

SLE nonspecific skin lesions

A

non scarring alopecia
mouth ulcers
photosensitivity
Raynaud’s phenomenon
deregulated IFN signaling
vasculitis/vasculopathy

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9
Q

neonatal lupus erythematosus

A

passive transfer of maternal IgG autoantibodies via placenta
diagnosed in pregnant females

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10
Q

what percentage of newborns with maternal autoantibodies develop NLE?

A

1%

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11
Q

clinical findings of NLE

A

cardiac
dermatologic
hematologic
hepatic

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12
Q

drug induced lupus associations

A

procainamide
hydralazine
Enbrel/remicade
minocycline
diltiazem
penicillamine
INH
quinidine

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13
Q

triggers of SLE?

A

excessive immune system activation
loss of tolerance of immune system
deregulated expression of components of immune system
deregulated estrogen metabolism

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14
Q

T cell lymphocytopenia

A

defect in switch from T helper 0 to T helper 2 cells
promotes B cell activation

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15
Q

environmental factors of lupus

A

UV light
viral infection (EBV, CMV)
hydralazine, INH, minocycline, anticonvulsants

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16
Q

immune dysregulation leading to SLE

A

hypergammaglobulinemia
complement deficiency
autoantibody production
cytokine activation
inability to clear immune complexes
organ/tissue deposition

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17
Q

malar

A

butterfly rash
symmetric
no scarring
SPARES nasolabial fold
1/2-1/3 onset

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18
Q

discoid

A

face, helix and scalp
asymmetric
demarcated papular lesions
scarring
alopecia

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19
Q

oral ulcerations are usually __________ and occur on the ___________

A

painless

hard palate or anywhere in buccal cavity

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20
Q

nose ulcerations occur on the _________ and can become a ___________

A

septum

asymmetric septal perforation

21
Q

immunologic criteria for SLE

A

antinuclear antibody
anti ds DNA
anti smith antibodies
antiphospholipid antibody
low complement
direct Coombs test

22
Q

individuals with identified genetic polymorphism are at ______________ for SLE compared to the general population

A

higher risk

23
Q

what is a genetic signature of SLE?

A

up regulation of genes induced by interferons

24
Q

In SLE, activation of complement and immune cells leads to

A

release of chemotaxins, cytokines, chemokine, vasoactive peptides and destructive enzymes

25
Q

what contributes to irreversible tissue damage of glomeruli, arteries, lungs, in SLE?

A

accumulation of growth factors and products of chronic oxidation

26
Q

key active treatment for SLE

A

hydroxychloroquine (Plaquenil)

27
Q

active treatments for SLE

A

hydroxychloroquine
biologics (BenLysta)
cytotoxics
NSAIDs
Steroids
topical steroids

28
Q

cytotoxics used to treat SLE

A

cellcept (mycophenolate)
cytoxan (cyclophos)
Imuran (azathioprine)
rituximab

29
Q

NSAIDs used to treat SLE

A

advil
mobic
naproxen

30
Q

steroids used to treat SLE

A

prednisone
medrol

31
Q

preventative treatment for SLE

A

sunscreen (at least SPF 30)
nutritional supplements (calcium, vit D, folate)

32
Q

Benlysta MOA

A

human monoclonal antibody
recognizes and blocks the biological activity of B-lymophocyte stimulator (BLyS)

33
Q

BLyS

A

naturally occurring protein
B cell survival factor

34
Q

elevated levels of BLyS _____________ of B cells which contribute to ______________

A

prolong the survival

production of autoantibodies

35
Q

beneficial side effects of Benlysta

A

reduce circulating CD19, CD20, naive and activated B cells
memory cells increased initially and slowly declined
reduce IgG and anti-ds DNA
increase in complement C3 and C4

36
Q

Hydroxychloroquine

A

anti malaria drug
antirheumatic

37
Q

hydroxycloroquine is thought to act as a ___________________ (as an anti rheumatic) and it inhibits the production of ______________

A

mild immunosuppressant

rheumatoid factor and acute phase reactants

38
Q

hydroxychloroquine accumulates in ___________ which stabilizes __________ and inhibits ______________

A

WBCs

lysosomal membranes

the activity of collegenases and proteases causing cartilage breakdown

39
Q

maximum effectiveness of hydroxychloroquine is

A

3-6 months

40
Q

half life of hydroxychloroquine

why is it this long?

A

537 hours (22.4 days)

extensive tissue uptake

41
Q

metabolites of hydroxychloroquine

which is major?

A

desethylhydroxychloroquine (DHCQ) - major
desethylchloroquine (DCQ)
bidesethylhydroxychloroquine (BDCQ)

42
Q

since hydroxychloroquine renal clearance does not correlate with creatinine clearance,

A

a dosage adjustment is not required for patients with renal impairment

43
Q

hydroxychloroquine is higher in what type of cell?

A

mononuclear

44
Q

what treatment for SLE is contraindicated in pregnancy?

A

cellcept (mycophenalate)

45
Q

which drug has to be a lower dose if the person with SLE is thiopurine methyltransferase (TPMT) deficient?

A

azathioprine (Imuran)

46
Q

which drug to treat SLE comes with a retinopathy risk?

A

Plaquenil

47
Q

which drug to treat SLE can cause infertility in women and men?

A

cyclophosphamide (Cytoxan)

48
Q

criteria for classification of SLE

A

SOAP BRAIN MD

serositis
oral ulcers
arthritis
photosensitivity
blood disorders
renal involvement
antinuclear antibodies
immunologic phenomena
neurologic disorder
malar rash
discoid rash