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Flashcards in Lupus Deck (25):
1

clinical manifestations of lupus

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2

criteria for lupus

4 or more of these criteria 

  • malar rash
  • discoid rash
  • photosensitivity
  • oral ulcers
  • arthritis
  • serositis
  • renal disorder
  • neuro disorder
  • sero/immunological disorder
  • pos ANA 

3

what interaction is essential for lupus autoantibody formation?

CD40-CD40L - "second signal" 

4

what do SLE autoantibodies do?

  • cell destruction (bind to RBCs and Plts)
  • cell "dysfuntion" (bind to vascular endothelium and neurons)

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5

why are intermediate-size immune complexes so problematic?

large enough to activate complement

small enough to evade RES

lupus pts get them and can't clear them

6

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

"the lupus band test"

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

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7

describe the rash in lupus

malar rash, discoid rash, photosensitivity

8

vascular problems in lupus

  • raynaud's w/ dilated nailfold capillaries
  • vasculitis and ischemia/infarct: from IC deposition in arterial walls --> inflammation and damage --> ischemia and infarct
    • digits, retina

9

describe typical lupus arthritis

PIP involvement w/ proliferative synovitis

10

lupus renal disease

extensive subendothelial deposits, scarred down glomerulus

full house staining 

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

11

serositis in lupus

immune complex deposition --> 

  • pericarditis w/ pericardial effusion
  • pleuritis w/ pleural effusion
  • peritonitis with ascites (less common)

12

SLE neurologic manifestiaons

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

+ vasculopathy in the brain

13

what is antiphospholipid syndrome?

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

14

what does antiphospholipid antibody do?

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

 

15

sequence of events in lupus

  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 

16

the most specific test for SLE

anti-ds-DNA

  • 20-70% in pts w/ lupus

 

17

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

glomerulonephritis!!

also some assoc w/ CNS disease

18

anti-smith antibody - what is it and utility?

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

19

what is the tx of choice for lupus GN?

systemic steroids and cyclophosphamide

20

compare side effects of steroids and cytoxan

steroids: medium side effects, everyone gets them

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

21

rituxan for SLE

B cell depleting antibody

lots of clinical trials, all w/ bad results

some rheums use anyway

22

BLyS

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

 

 

23

late musculoskeletal complications of SLE

  • Osteonecrosis
  • Osteoporosis

24

important long term secondary problem in lupus

CAD: assoc w/ duration of disease

25

Benlysta/Belimumab

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