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IBI Test 2 > B3.020 Big Case Systemic Lupus Erythematosus > Flashcards

B3.020 Big Case Systemic Lupus Erythematosus Flashcards

1
Q

what is one of the most common presenting symptoms of lupus?

A

non destructive arthritis

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2
Q

what does it mean to be “non destructive”?

A

can straighten out joints that are deformed without pain

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3
Q

Jaccoud’s arthropathy

A

ligament laxity

can look like deformity is present, but can correct it on exam

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4
Q

describe serositis

A

pleural effusion
pericardial effusion
pleurisy/pleuritic chest pain

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5
Q

what is one way to check for serositis?

A

ultrasound of the heart to check for surrounding fluid

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6
Q

characterize oral ulcers associated with lupus

A

can be painless

can involve hard palate

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7
Q

what are three primary cutaneous clinical manifestations of lupus

A

Malar “butterfly” rash
subacute cutaneous lupus rash
discoid lupus rash

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8
Q

describe the butterfly rash

A

spares nasal labial fold

get the rash a few days after being in the sun

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9
Q

hematologic manifestations of lupus

A

anemia
thrombocytopenia
leukopenia
-can be secondary to lupus, related to comorbidities, or treatment for lupus

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10
Q

renal manifestations of lupus

A

glomerulonephritis (lupus nephritis)
multiple different mechanisms of actions
symptoms include: hematuria, proteinuria, hypertension, edema

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11
Q

describe the connection between lupus nephritis and type 3 hypersensitivity

A
  • consequence of soluble antigen-antibody complexes form in blood and deposit in the tissues
  • IgG containing immune complexes activate complement in tissues leading to damage (punches holes in basement membrane)
  • consumption of C3 and C4 which can be measured in blood
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12
Q

cardiac manifestation of lupus

A

pericarditis
Libman-Sack’s endocarditis
-noninfectious vegetations

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13
Q

what are noninfectious vegetations

A

can emobolize off an cause stroke, etc.

very different treatment options from infectious endocarditic, but cant always be differentiated

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14
Q

neurological manifestations of lupus

A

seizures

psychosis

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15
Q

what is synovitis

A

inflammation and swelling in joints

not specific to lupus

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16
Q

what is an antinuclear antibody

A

antibody with specificity to components of nucleus

indirect immunofluorescence is the preferred technique of detection

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17
Q

describe the process of ANA indirect immunofluorescence

A
  1. add patient plasma to microscope slide with attached cell
  2. wash to eliminate unattached antibodies
  3. add fluorescent tagged anti-human antibody
  4. assess if fluorescent signal present, continue until lowest dilution is still positive
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18
Q

how is the ANA indirect immunofluorescence test reported

A

reported as a titer which reflects dilution where result is still positive
start at 1:40 then 1:80 then 1:160

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19
Q

what are some limitations of ANA testing

A

positive in up to 15% of general population
variability in results between assays
quantitative value does not have clinical significance (does not trend with disease activity)

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20
Q

is ANA sensitive?

A

yes

few false negatives

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21
Q

is ANA specific?

A

no

high false positive

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22
Q

discuss anti-double stranded DNA antibodies

A

very specific for lupus
felt to be pathogenic
trend with disease activity in contrast to other antibodies

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23
Q

what are other specific antinuclear antibodies used in lupus diagnosis?

A

anti-smith
anti-RNP
anti-SSA (Ro)
anti-SSB (La)

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24
Q

what are antiphospholipid antibodies associated with?

A

thrombosis

pregnancy loss

25
Q

what are some functional assays of antiphospholipid antibodies?

A

Lupus anticoagulant
Dilute Russell Viper Venom Test
prolonged PTT

26
Q

what are some other antiphospholipid antibodies that can be tested?

A

anti-cardiolipin IgM and IgG

anti-beta 2 glycoprotein 1 IgM and IgG

27
Q

how is the complement system associated with lupus?

A

activation of the classical pathway is associated with flares of lupus disease activity
leads to decline in C1-4

28
Q

describe the classical complement pathway

A

initiated by Ag-Ab complexes (only IgG and IgM participate)
C1q binds to Ch
recruits C1r and C1s to form C1qr2s2 to cleave C2 and C4 to produce C4b2a (classic C3 convertase)

29
Q

what types of medication can cause drug induced lupus?

A

hydralazine and procainamide (old cardiac drugs, not used much anymore)
Isoniazid (TB)
minocycline (used for acne treatment)

30
Q

when do patients develop ANA and other autoantibodies relative to the diagnosis of lupus?

A

autoantibodies precede diagnosis by years

31
Q

what is the acronym for ACR classification criteria

A 
S-serositis
O- oral ulcers
A- arthritis
P- photosensitivity
M- malar rash
D- discoid rash
B- blood abnormalities
R- renal abnormalities
A- positive ANA
I- immunologic abnormalities (dsDNA, Smith, cardiolipin)
N- neurologic
32
Q

what is drug induced lupus

A

associated with histone antibody
most common manifestations are arthritis and pleurisy
very unusual to have severe involvement such as renal or CNS involvement

33
Q

can you have just cutaneous lupus?

A

yes

discoid or subacute cutaneous

34
Q

discuss the epidemiology of lupus

A 
20-150 cases per 100,000
more common in women than men
15:1 women:men
most commonly diagnosed between 16-55
ratio is lower in pediatric population and post-menopausal population
35
Q

what races are more at risk for lupus?

A

Asians, African americans, African caribbeans, Hispanic americans

36
Q

what are some suspected contributors to the pathogenesis of lupus?

A 
genetics (gene mutations, epigenetics)
hormones (female predominance)
environmental factors (UV light)
37
Q

what is the risk of lupus in identical twins?

A

14-57%

38
Q

what are some categories of genetic polymorphisms that confer higher risk of lupus?

A

complement
DNA degradation
antigen presentation
innate immunity/ interferon alpha

39
Q

what are 3 contributors to the development of lupus

A
  1. inefficient clearance of apoptotic debris
  2. breach of tolerance
  3. immune complex deposition and complement activation
40
Q

why would inefficient clearance of apoptotic debris lead to lupus?

A

persistent cellular debris increases likelihood of the body creating antibodies against it

41
Q

what are the 5 and 20 year survival rates of lupus?

A

> 90% and 80% respectively

42
Q

what is the leading cause of death in lupus patients?

A

cardiovascular disease (heart attacks)

43
Q

what types of diseases are lupus patients at a higher risk of death from?

A

renal
cardiovascular
infection

44
Q

why are lupus patients more at risk of infections?

A

immunosuppressant medications in addition to an already deficient immune system

45
Q

what is the target of hydroxychloroquine therapy?

A

interfere with antigen processing and as a result peptide-MHC protein complexes

46
Q

how does hydroxychloroquine interfere with antigen presentation?

A

multiple mechanisms
interfering with lysosome acidification
prevention of TLR binding epitopes

47
Q

what is the target of Belimumab (Benlysta)?

A

autoantibody production/ B cell function

48
Q

how does Belimumab interfere with B cell function?

A

blocks the binding of soluble B-cell activating factor (BAFF)
also known as BLys (B lymphocyte stimulator)

49
Q

what is the target of Azathioprine (Imuran)?

A

decrease function of B and T cells

50
Q

how does Azathioprine decrease function of B and T cells?

A

pure analog which inhibits purine synthesis leading to inhibition of DNA and RNA
results in reduced cellular proliferation, particularly leukocytes

51
Q

how does Mycophenolate mofetil (CellCept) decrease function of B and T cells?

A

inhibits inosine monophosphate dehydrogenase which leads to reduced guanine monophosphate for purine synthesis in the de novo pathway used in proliferation of B and T cells

52
Q

how does Cyclophosphamide (Cytoxan) decrease the function of B and T cells?

A

phosphoramide mustard attaches an alkyl group to DNA which ultimately leads to cell apoptosis
traditional chemotherapy agent

53
Q

discuss corticosteroid therapy for lupus

A

multiple mechanisms of action (genomic and nongenomic)

may inhibit interferon alpha

54
Q

what are some consequences of corticosteroids?

A 
weight gain
decline in bone health
DM
infection risk
mood (psychosis)
55
Q

what meds are contraindicated in the situation of a potential pregnancy?

A

cyclophosphamide

mycophenolate mofetil

56
Q

what tis the typical function of steroid treatment for lupus?

A

a bridge until the relief of the other drug is systemic

not a maintenance agent

57
Q

what are some non-pharmacological treatments of lupus?

A 
sun avoidance
contraception
high risk for CAD (exercise, low threshold to treat cholesterol)
advise nicotine cessation
immunizations
screen for depression
58
Q

what cues can help distinguish an infection from a lupus flare?

A

no worsening of other lupus symptoms
complements improving compared to last visit
CRP elevations (more typical of infection than lupus flare)

59
Q

what prevents CRP elevation in a lupus flare?

A

IFNa

IBI Test 2 (10 decks)

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