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Systemic Lupus
ErythematosusSteve Beesley
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History
1948 Malcolm Hargraves discovers the
lupus erythematosus (LE cell!
19"# $he %irst a&ti')* a&ti+ody is
ide&ti%ied!
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LE Cell
$he LE cell is a&eutrophil that hase&gul%ed
thea&ti+ody'coated
&ucleus o% a&other&eutrophil! LE cells may
appear
i& rosettes ,herethere are several&eutrophils vyi&g
%ora& i&dividualcompleme&t coveredprotei&!
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Genetic Associations
HL*-s are loci o& ge&es that code
%or certai& . chai& o& the MH/
comple0
HL*'2
HL*'3
HL*'B1 5&volved i& mediati&gproductio& o%
a&ti+odies to ds')*
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Symptoms
)o&'speci%ic6
7atigue
eight loss
Malaise ge&erally %eeli&g ill
7ever
*&ore0ia (over time
*rthritis
9:; o% patie&ts e0perie&ce arthritic symptoms
Symmetrical
*ppears i& ha&ds< ,rists< a&d =&ees
mai&ly
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Skin Manifestations
Malar or Butter%lyash
iscoid ash
Stimulated +y >?light
S=i&ma&i%estatio&s
o&ly appear i& 3:'4:; o% lupuspatie&ts!
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Renal (Kidney)
Manifestations ":'#:; o% all lupus
patie&ts e0perie&ce re&aldevelopme&ts!
Most a&gerous6
@lomerulo&ephritis
,here at least ":; o% theglomeruli have
cellularproli%eratio&
@lomeruli capillary+eds i& the =id&ey that%ilter the
+lood!
e&al 7ailure +ecause o%@lomerulo&ephritis is
theleadi&g cause o% deathamo&g lupus patie&ts!
)ormal
@lomerulo&ephritis
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ther Manifestations
/ardiac
/e&tral )ervous System
Hematological
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Main !atholo"y
$he plasma cells are produci&g
a&ti+odies that are speci%ic %or sel%
protei&s< &amely ds')*
Averactive B'cells
Suppressed regulatory %u&ctio& i& $'cells
Lac= o% $'cells
*ctivatio& o% the /ompleme&t system
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#eracti#e $%cells
Estroge& is a stimulator o% B'cell activity
Lupus is much more prevale&t i& %emales o%
ages 1"'4"
Height o% Estroge& productio&
5L'1:< also a B'cell stimulator is i& high
co&ce&tratio& i& lupus patie&t serum!
High co&ce&tratio& li&=ed to cell damage
caused +y i&%lammatio&
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&%cell Malfunctions
7c regio& s,itch
CD
Leads to mal%u&ctio& i& sig&ali&g
a&ddecreased 5L'2 productio&
5&creased levels o% /a2E
Leads to spo&ta&eous apoptosis
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&%cell Si"nal &ransduction
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Acti#ation of Complement
System
/ompleme&t system is activated +y
the +i&di&g o% a&ti+odies to %oreig&
de+ris!
5& this case its over activatio&
B/s lac= /1 receptor
ecreasi&g the a%%ective removal o%
comple0es
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'"G !atho"en
5g@ is the most Fpathoge&icG
+ecause it %orms i&termediate sied
comple0es that ca& get to the small
places a&d +loc= them!
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A is the Main man
)* is the mai& a&tige& %or ,hich
a&ti+odies are %ormed!
E0tracellular )* has a& a%%i&ity %or+aseme&t
mem+ra&e ,here it is
+ou&d +y autoa&ti+odies!
/lassical thic=e&i&g o% the +aseme&tmem+ra&e
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&estin"
ES
>ri&alysis
/ompleme&t $est
$ests levels o% /3< /4< /H":
Lo, levels i&dicates possi+le prese&ce o%
disease
7*)* 7luoresce&t a&ti&uclear a&ti+ody
Auchterlo&y $est sho,s i&teractio&s
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*AA
EL5S* $est
@e&erally test %or6
ds')* a&ti+odies
*&tihisto&ea&ti+odies
Bi&ds to )*sed to determi&e
immu&ological
speci%icity
ules out a %alsepositive
Sho,s the serum
does or does &othave a&ti&uclear
a&ti+odies
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Summary
Lupus *utoimmu&ity
Systemic a&d a%%ects co&&ective tissue
/aused +y mal%u&ctio&s o%6
$'cells
B'cells
/ompleme&t System
Sig&al $ra&sductio& /a& +e lethal or &ot
>&iKue to each i&dividual
