Lupus (exam 1) Flashcards

(48 cards)

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

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
what contributes to irreversible tissue damage of glomeruli, arteries, lungs, in SLE?
accumulation of growth factors and products of chronic oxidation
26
key active treatment for SLE
hydroxychloroquine (Plaquenil)
27
active treatments for SLE
hydroxychloroquine biologics (BenLysta) cytotoxics NSAIDs Steroids topical steroids
28
cytotoxics used to treat SLE
cellcept (mycophenolate) cytoxan (cyclophos) Imuran (azathioprine) rituximab
29
NSAIDs used to treat SLE
advil mobic naproxen
30
steroids used to treat SLE
prednisone medrol
31
preventative treatment for SLE
sunscreen (at least SPF 30) nutritional supplements (calcium, vit D, folate)
32
Benlysta MOA
human monoclonal antibody recognizes and blocks the biological activity of B-lymophocyte stimulator (BLyS)
33
BLyS
naturally occurring protein B cell survival factor
34
elevated levels of BLyS _____________ of B cells which contribute to ______________
prolong the survival production of autoantibodies
35
beneficial side effects of Benlysta
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
Hydroxychloroquine
anti malaria drug antirheumatic
37
hydroxycloroquine is thought to act as a ___________________ (as an anti rheumatic) and it inhibits the production of ______________
mild immunosuppressant rheumatoid factor and acute phase reactants
38
hydroxychloroquine accumulates in ___________ which stabilizes __________ and inhibits ______________
WBCs lysosomal membranes the activity of collegenases and proteases causing cartilage breakdown
39
maximum effectiveness of hydroxychloroquine is
3-6 months
40
half life of hydroxychloroquine why is it this long?
537 hours (22.4 days) extensive tissue uptake
41
metabolites of hydroxychloroquine which is major?
desethylhydroxychloroquine (DHCQ) - major desethylchloroquine (DCQ) bidesethylhydroxychloroquine (BDCQ)
42
since hydroxychloroquine renal clearance does not correlate with creatinine clearance,
a dosage adjustment is not required for patients with renal impairment
43
hydroxychloroquine is higher in what type of cell?
mononuclear
44
what treatment for SLE is contraindicated in pregnancy?
cellcept (mycophenalate)
45
which drug has to be a lower dose if the person with SLE is thiopurine methyltransferase (TPMT) deficient?
azathioprine (Imuran)
46
which drug to treat SLE comes with a retinopathy risk?
Plaquenil
47
which drug to treat SLE can cause infertility in women and men?
cyclophosphamide (Cytoxan)
48
criteria for classification of SLE
SOAP BRAIN MD serositis oral ulcers arthritis photosensitivity blood disorders renal involvement antinuclear antibodies immunologic phenomena neurologic disorder malar rash discoid rash