Lupus Flashcards

1
Q

clinical manifestations of lupus

A
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

criteria for lupus

A

4 or more of these criteria

  • malar rash
  • discoid rash
  • photosensitivity
  • oral ulcers
  • arthritis
  • serositis
  • renal disorder
  • neuro disorder
  • sero/immunological disorder
  • pos ANA
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

what interaction is essential for lupus autoantibody formation?

A

CD40-CD40L - “second signal”

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

what do SLE autoantibodies do?

A
  • cell destruction (bind to RBCs and Plts)
  • cell “dysfuntion” (bind to vascular endothelium and neurons)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

why are intermediate-size immune complexes so problematic?

A

large enough to activate complement

small enough to evade RES

lupus pts get them and can’t clear them

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

how do you detect immune complexes in skin biopsy of a lupus pt?

A

“the lupus band test”

anti-human antibody tagged w/ FITC (green flourescent dye - binds to pt’s antibody in dermal-epidermal junction

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

describe the rash in lupus

A

malar rash, discoid rash, photosensitivity

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

vascular problems in lupus

A
  • raynaud’s w/ dilated nailfold capillaries
  • vasculitis and ischemia/infarct: from IC deposition in arterial walls –> inflammation and damage –> ischemia and infarct
    • digits, retina
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

describe typical lupus arthritis

A

PIP involvement w/ proliferative synovitis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

lupus renal disease

A

extensive subendothelial deposits, scarred down glomerulus

full house staining

types: membranous lupus nephritis, mesangial proliferatie glomerulonephritis, membranoproliferative lupus nephritis, diffuse proliferative lupus nephritis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

serositis in lupus

A

immune complex deposition –>

  • pericarditis w/ pericardial effusion
  • pleuritis w/ pleural effusion
  • peritonitis with ascites (less common)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

SLE neurologic manifestiaons

A
  • psychosis
  • seizures
  • stroke
  • cognitive function abnormalities
  • transverse myelopathy
  • mononeuritis multiplex
  • organic brain syndrome

+ vasculopathy in the brain

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

what is antiphospholipid syndrome?

A

occurs in 25-50% of SLE pts (can also happen in non-lupus pts)

2 important tests: lupus anticoagulant & anticardiolipid antibody

sx: thromboiss, recurrent or late pregnancy loss, thrombocytopenia
tx: warfarin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

what does antiphospholipid antibody do?

A

antibody (usually IgG) binds Beta-2-glycoprotein domain I, which is part of the natural immune system

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

sequence of events in lupus

A
  1. a break in tolerance to self-antigens
  2. production of autoantibodies to self
  3. formation of immune complexes
  4. difficulty clearing ICs
  5. tissue damage: 3 causes
    1. autoantibodies against target tissue
    2. autoantibodies directly causing “dysfunction”
    3. indirect innocent bystander organ damage
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

the most specific test for SLE

A

anti-ds-DNA

  • 20-70% in pts w/ lupus
17
Q

positive anti-ds-DNA is highly assoc w/ what complication?

A

glomerulonephritis!!

also some assoc w/ CNS disease

18
Q

anti-smith antibody - what is it and utility?

A
  • antibody to small nuclear ribosomal protein
  • seen in 20-30% pts
  • does not exist in other diseases - not sensitive, but very specific!!!
19
Q

what is the tx of choice for lupus GN?

A

systemic steroids and cyclophosphamide

20
Q

compare side effects of steroids and cytoxan

A

steroids: medium side effects, everyone gets them

cytoxan side effects: very bad, but not many people get them (cytopenias, malignancy)

21
Q

rituxan for SLE

A

B cell depleting antibody

lots of clinical trials, all w/ bad results

some rheums use anyway

22
Q

BLyS

A
  • B cell survival factor
  • produced by immune cells
  • soluble form is active
  • trimeric
23
Q

late musculoskeletal complications of SLE

A
  • Osteonecrosis
  • Osteoporosis
24
Q

important long term secondary problem in lupus

A

CAD: assoc w/ duration of disease

25
Q

Benlysta/Belimumab

A

mAb targeting BLyS - recently approved drug. so far tx effect is mild

  • does not work for black pts
  • pts w/ nephritis/CNS disease excluded from trial - focused on skin & arthritis tx