SLE

Question 0

when does SLE typically prsent

Answer 0

20-30 years

Question 1

hallmark of SLE

Answer 1

production of autoantibodies; prototypical immune complex disease

Question 2

what causes lupus-like disease?

Answer 2

hereditary complement deficiency

Question 3

what organs can SLE effect?

Answer 3

renal
pulmonary
cardiac
skin

(one often predominantes)

Question 4

non-specific symptoms in almost all patients

Answer 4

fatigue (80-100%)
fever>80% in patients with active disease
MSK dz: all patients (mostly PIP or MCP, knees, wrists, but any)

Question 5

to classify lupus you need greater than or equal to ( ) symptoms

Answer 5

4

Question 6

4 skin aspects

Answer 6

malar rash
discoid rash
photosensitivty
oral ulcer

Question 7

2 types of serositis

Answer 7

pleuritis

pericarditis

Question 8

two theroies of how autoantibodies are generated

Answer 8

non-specific polyclonal b cell activation

normal immune response, but with some CD4+ t cell responding to autoantigen on MHCII (first signal–second signal co-stim molecules)

Question 9

mice treated with Cd4 depleting or Cd40/CD40L blocking antibodies..

Answer 9

recovered frorm lupus and survived better than untreated mice

Question 10

once the antibodies are generated…

Answer 10

bind directly to cells–>cell dysfunction, cell destruction (anemia, thrombocytopenia)
form immunocomplexes to get stuck places

Question 11

another issues with immunecomplexes

Answer 11

medium sized therefore large enough to activate Complement, but small enough to evade the reticuloendothelial system

Question 12

malar rash

Answer 12

fized erythema, flat or raised sparing nasolabial folds

Question 13

discoid rash

Answer 13

raised patches, adherent kerotoc scaling

Question 14

RaYNAUDS

Answer 14

induced by cold or emotion–change in skin color–white–>blue–>red
digital infarcts, dry gangrene

Question 15

arthropathy

Answer 15

reversible once SLE is controlled without and permanent damage

Question 16

Jaccoud’s arthropathy

Answer 16

hand deformity rare in SLE, but Jaccoud’s that happens- still different frorm RA in that only tendons and ligaments are affected, so patients can actually correct their hand shape

Question 17

renal disease in lupus

Answer 17

extensive subendothelial deposits and everywhere else
full house pattern
wire looping

Question 18

end result of renal disease in lupus

Answer 18

glomerular degeneration and fibrosis

Question 19

libman sachs endocarditis

Answer 19

pereicardial effusion leads to vegetations on mitral valve

Question 20

types of serositis

Answer 20

pleural effusions-loss of costophgrenic angle
pericardial effusion-libman sachs
peritonitis-less common

Question 21

usual SLE CNS

Answer 21

bland vasculopathy

Question 22

APS

Answer 22

can occur in presence or absence of lupus

Question 23

APL antibodies

Answer 23

seen in 25-50% of SLE patients, usually intermittently and at low titer

Question 24

2 important APL tests

Answer 24

lupus anticoag- interferes with clotting and increases PTT (not corrected by adding plasma) anti-cardiolipin ab- targets cardiolipin, an importnat out mito membrane protein

Question 25

major antigen recognized by APA is

Answer 25

B2 glycoprotein (B2GP1)-apolipoprotein H (member of C cascade that may be involved in homeostatic regulation)

Question 26

non-pathological abs are

Answer 26

IGM and target domain V of BGP

Question 27

pathological ABS

Answer 27

domain I-Igg

Question 28

APS shows with

Answer 28

thrombosis recurrent fetal loss in late trimester thrombocytopenia livedo reticularis

Question 29

treatment of APS

Answer 29

high ontensity warfrain (INR 3-4)

Question 30

3 serological tests for lupus

Answer 30

ANA anti-dsDNA anti-Smith

Question 31

ANA

Answer 31

``` diffuse or peripheral rim pattern high sens (almost all SLE patients have it) but low spec (present in patients with other disease) *except centromere pattern in specific to CREST ```

Question 32

anti-dsDNA

Answer 32

20-70% SLE | most specific to SLE, esp GNitis

Question 33

anti-smith (antisRNA)

Answer 33

only 20-30% of SLE patients, but not seen in any other disease (high spec)

Question 34

treatment of lupus depends on

Answer 34

clinical manigestation

Question 35

articular sx

Answer 35

NSAID, COX2 inhibs low dose steroids plaquenil dapsone

Question 36

serositis

Answer 36

NSAIDS and COX2 inhib | cytotoxin

Question 37

cutaneous disease

Answer 37

topical steroids plaquenil systemic cytotoxins dapsone

Question 38

heme disease tx

Answer 38

steroids immunosuppressive rituximab

Question 39

renal tx

Answer 39

systemic steroids cyclophosphamidde --good treatment but sideeffects too

Question 40

CNS tx

Answer 40

high dose steroids cytotoxic antipsychotics

Question 41

future rx directions

Answer 41

Benlysta

Question 42

Benlysta

Answer 42

inhibits binding of soluble blys to R on b cell mainly helps skin and arthritis, no nephro or CNS disease *only mildly helpful

Question 43

late complications in SLE

Answer 43

osteonecrosis (avascular b necrosis) osteoporosis-lumbar spine early atherosclerosis pregnancy (APS major factor)--loss in 50% of pregnancies