SLE Flashcards
when does SLE typically prsent
20-30 years
hallmark of SLE
production of autoantibodies; prototypical immune complex disease
what causes lupus-like disease?
hereditary complement deficiency
what organs can SLE effect?
renal
pulmonary
cardiac
skin
(one often predominantes)
non-specific symptoms in almost all patients
fatigue (80-100%)
fever>80% in patients with active disease
MSK dz: all patients (mostly PIP or MCP, knees, wrists, but any)
to classify lupus you need greater than or equal to ( ) symptoms
4
4 skin aspects
malar rash
discoid rash
photosensitivty
oral ulcer
2 types of serositis
pleuritis
pericarditis
two theroies of how autoantibodies are generated
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)
mice treated with Cd4 depleting or Cd40/CD40L blocking antibodies..
recovered frorm lupus and survived better than untreated mice
once the antibodies are generated…
bind directly to cells–>cell dysfunction, cell destruction (anemia, thrombocytopenia)
form immunocomplexes to get stuck places
another issues with immunecomplexes
medium sized therefore large enough to activate Complement, but small enough to evade the reticuloendothelial system
malar rash
fized erythema, flat or raised sparing nasolabial folds
discoid rash
raised patches, adherent kerotoc scaling
RaYNAUDS
induced by cold or emotion–change in skin color–white–>blue–>red
digital infarcts, dry gangrene
arthropathy
reversible once SLE is controlled without and permanent damage
Jaccoud’s arthropathy
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
renal disease in lupus
extensive subendothelial deposits and everywhere else
full house pattern
wire looping
end result of renal disease in lupus
glomerular degeneration and fibrosis
libman sachs endocarditis
pereicardial effusion leads to vegetations on mitral valve
types of serositis
pleural effusions-loss of costophgrenic angle
pericardial effusion-libman sachs
peritonitis-less common
usual SLE CNS
bland vasculopathy
APS
can occur in presence or absence of lupus
APL antibodies
seen in 25-50% of SLE patients, usually intermittently and at low titer
2 important APL tests
lupus anticoag- interferes with clotting and increases PTT (not corrected by adding plasma)
anti-cardiolipin ab- targets cardiolipin, an importnat out mito membrane protein
major antigen recognized by APA is
B2 glycoprotein (B2GP1)-apolipoprotein H (member of C cascade that may be involved in homeostatic regulation)
non-pathological abs are
IGM and target domain V of BGP
pathological ABS
domain I-Igg
APS shows with
thrombosis
recurrent fetal loss in late trimester
thrombocytopenia
livedo reticularis
treatment of APS
high ontensity warfrain (INR 3-4)
3 serological tests for lupus
ANA
anti-dsDNA
anti-Smith
ANA
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
anti-dsDNA
20-70% SLE
most specific to SLE, esp GNitis
anti-smith (antisRNA)
only 20-30% of SLE patients, but not seen in any other disease (high spec)
treatment of lupus depends on
clinical manigestation
articular sx
NSAID, COX2 inhibs
low dose steroids
plaquenil
dapsone
serositis
NSAIDS and COX2 inhib
cytotoxin
cutaneous disease
topical steroids
plaquenil
systemic cytotoxins
dapsone
heme disease tx
steroids
immunosuppressive
rituximab
renal tx
systemic steroids
cyclophosphamidde
–good treatment but sideeffects too
CNS tx
high dose steroids
cytotoxic
antipsychotics
future rx directions
Benlysta
Benlysta
inhibits binding of soluble blys to R on b cell
mainly helps skin and arthritis, no nephro or CNS disease
*only mildly helpful
late complications in SLE
osteonecrosis (avascular b necrosis)
osteoporosis-lumbar spine
early atherosclerosis
pregnancy (APS major factor)–loss in 50% of pregnancies
