AUTOIMMUNE DZ

DISEASE EPIDEMIOLOGY/
PATHOGENESIS
CLINICAL LAB PATHOLOGY
SCLERODERMA 1)Excess collagen deposition:
Oe!p!od"ction #$ %i#!o#lasts &alte!ed
gene exp!ession)
C$to'ines( l$)p*o'ines+ alte!ed
ECM gene exp!ession
T,- &)onoc$tes( l$)p*oc$tes(
platelets)+ sti)"lated collagen( ECM
p!od"ction
.) Capilla!$( s)all aa lesions
Ea!l$ In d/
0ac"oli/ation( s1elling( nec!osis(
slo"g*ing o% endot*elial cells(
!ed"plication o% 2M( s"#endot*elia
acc")"lation o% loose CT( int!aasc3
platelet t*!o)#osis
4e!iasc"la! cell in%ilt!ate: T5cell( )acs
6) 7")o!al( cell"la! i))"nit$
alte!ations
Extent o% in%ilt!ation8see!it$(
p!og!ession
L$)p*oc$tes &T7)5M7C II5DR( )acs
9:; &<:1)
<=5>=$?o
1)Ea!l$
Raynauds: Cold5e)otion ind"ced const!iction o% digital a!te!ioles
5p!ecede )os to $!s
4"%%$ *ands?%o!ea!)s
A!t*!algia( palpa#le tendon %!iction !"# &1!ists?an'les)
Di%%"se *$pe!pig)entation &@salt A peppe!B)C p!"!itis
.)C"taneo"s
Skin scle!sis: T*ic'ened( *ide#o"nd s'in &distal+ p!oxi)al)D ta"t(
s*in$( tig*tl$ ad*e!ent
1idening o% c"ticles( loss o% so%t tiss"e at %inge!tips
Loss o% 1!in'les( 2ea'ed nose( t*in lips( tig*tl$ p"sed( !adial
%"!!o1ing( Mic!osto)ia
Telangiectasia: Dilated capilla!$ loops5*ands( %ace( lips( tong"e( ,I
#leeding
4ain%"l !ec"!!ent "lce!ations5%inge!tips
C"taneo"s calci%ication: Calci") apatite at %inge!tips( extenso!
%o!ea!)s
6)S'eletal
S$))et!ical pol$a!t*!algia( cont!act"!e o% %inge!s &%i#!osis)( ca!pal
t"nnel( coa!se leat*e!$ c!epit"s &tendon %!iction !"#)( ac!o5osteol$sis(
indolent )$opat*$ &)) %i#!osis( %i#e! at!op*$)
<),I
Esop*ageal a#n &LES inco)petence( E pe!istalsis)( d$sp*agia(
od$nop*agia( ,ERD( *oa!se( co"g*( asp3 pne")oD 2a!i")
esop*og!a)D )ano)et!$ to !?o pol$)$ositisD ps"edodie!tic"la
>)Res"ia#!y$ Leadin% cause !& M'M
4"l)ona!$ %i#!osis5CT5g!o"nd glass patte!n in lo1e! lo#es
&exe!tional d$spnea( li)3 exe!cise)( p"l)3 7TN &E di%%"sing capacit$(
Dopple!)( co! p"l)onaleD 4"l) %xn st"dies
F)Renal$ M!s# deadly co)plication &1>5.=G)
A#!"pt onset( Malignant 7TN57?A( !etinopat*$( ac"te ca!diac
%ail"!e( MI( st!o'e( olig"!iaD HHplas)a !enin
I)7ea!t &J=G): Tac*$a!!$t*)ia( cond"ction dist"!#ance &1ide KRS(
A0 #loc')( pe!ica!dial e%%"sion
J)4e!ip*e!al ne"!opat*$( CNS spa!ed( e!ectile d$s%xn( sicca(
No!)al ESR
ANA &C) :L=G
Anti5DNA?S) &5)
Anti5'inetoc*o!e &C) :J=G
1?li)ited SSc
An#i(Scl()*$ Di&&use SSc
An#i(cen#!+ee$ Li+i#ed
ANA&C) in spec'led?n"cleola!
patte!n 1?Anti5Scl5I=&5)( Anti5
cent!o)e!e &5) + See!e( !apid(
p!og!essie( 1?ea!l$ isce!al
incole)ent
HH 4las)a !enin: I)pending
!enal c!isis
S'in #iops$
T*inning o% epide!)is
T*ic'ened de!)is
,, c!lla%en
Loss o% no!)al de!)al
appendages( )onon"clea!
in%la))3 cell in%ilt!ates5lo1e!
de!)is
L"ng: Inte!stitial %i#!osis(
occl"sion o% asc3 l")en
Renal: Malignant 7TN5inti)al
p!oli%3 o% inte!lo#"la!( a!c"ate
aaD %i#!inoid nec!osis o% )ediaD
no!)al glo)e!"li
DISEASE EPIDEMIOLOGY/
PATHOGENESIS
CLINICAL LAB PATHOLOGY
*$pot*$!oid &)ild)( 42C
SMSTEMIC
LU4US
ERMT7EMATOSI
S
A"toi))"ne d/
P!duc#i!n !& Au#!A- &ANA)(
i))"ne co)plexes( acti3 o%
co)ple)ent
7LA5DR.( 7LA5DR6
I+"aied cleaance o% ci!c"lating
i))"ne co)plexes .
o
to E CR1(
de%ectie -c5R( de%3 o% C<A
C!ite!ia: < si)"ltaneo"s o! se!iall$
1) Mala! !as*: Spa!ing o% nasola#ial %olds
.)Discoid !as*: inne! pinnae( not p*otosensitieD %ollic"la! pl"gging(
s'in at!op*$( scaling( telangiectasia( s'in e!$t*e)a
6)4*otosensitiit$: s'in
Alopecia: Ree!si#le *ai! t*inning
Ra$na"dNs
<)O!al( nasop*a!$ngeal "lce!s: 7a!d( so%t palate( nasal sept")D
painless
>)A!t*!itis: N!ne!si.e o% O. Points
F)Se!ositis: 4le"!itis( pe!ica!ditis
I)Renal diso!de!: 4!otein"!ia :=3>g?d o! 6C o! cell3 casts
J)Ne"!ologic: Sei/"!e( ps$c*osis
L)7e)atologic: 7e)ol$tic ane)ia( Q2CR<===( platelets R1==(===
1=) I))"nologic: ds5DNA( Anti5S)( Antip*osp*olipid( l"p"s
anticoag3( %alse &C) R4R
H incidence o% at*e!oscle!otic *ea!t d/ in SLE &p!e)enopa"sal 9):
MaPo! ca"se o% deat*
ANA/01$ 1==G #"t it is not
diagnostic
Seu+ An#i(DNA A-: Ma!'e!
%o! deelop)ent o% !enal d/
2 C!+"le+en#: Actie !enal d/
SSO,RENNS
SMNDROME
.
nd
)ost co))on a"toi))"ne
!*e")atic d/
Middle aged elde!l$ 1*ite 9
9:; &L:1)
4!i)a!$ SPog!enNs T 1*en t*e a#oe
s$nd!o)e is not associated 1it*
anot*e! a"toi))"ne disease
Seconda!$ SPog!enNs T 1*en t*e a#oe
s$nd!o)e is associated 1it* anot*e!
a"toi))"ne disease3 &RA( SLE(
p!i)a!$ #ilia!$ ci!!*osis( scle!ode!)a(
pol$)ositis( 7as*i)otoNs t*$!oiditis(
inte!stitial p"l)ona!$ %i#!osis)3
C!ite!ia:
1) Oc"la! s$)pto)s: d!$ e$es( %o!eign #od$ sensation in e$es( "se
o% l"#!icants
.) O!al s$)pto)s: d!$ )o"t*( s1ollen salia!$ glands( %!eU"ent
%l"id inta'e 1it* %ood3
6) Oc"la! signs: Sc*i!)e!Ns o! Rose #engal test3
<) C*a!acte!istic *istopat*olog$: %oc"s sco!e:1 &la#ial )ino!
salia!$ gland #iops$5gold std)
>) Salia!$ gland inole)ent doc")ented #$ scintig!ap*$(
sialog!ap*$( o! salia!$ %lo13
F) A"toanti#odies: R-( ANA( o! anti5Ro&SSA) o! anti5La&SS2)
Sicca s$nd!o)e: Di)inis*ed lac!i)al A salia!$ sec!etion
3ea#!c!n4uci#i.i#is sicca: D!$ e$es( %o!eign #od$ sensation in t*e
e$es &li'e di!t #le1 in $o"! e$es)( #"!ning( itc*ing( !edness( %atig"e(
)"coid disc*a!ge( p*otosensitiit$( #l"!!ed ision( oc"la! %ila)ents
&%ila)enta!$ 'e!atitis)3
5e!s#!+ia: D!$ )o"t*( o!al disco)%o!t( inc!eased ingestion o%
liU"ids( &1ate! #ottle sign)( di%%ic"lt$ spea'ing( alte!ed taste(
di%%ic"lt$ s1allo1ing( di%%ic"lt$ c*e1ing( ad*e!ence o% %ood to
#"ccal )"co"s )e)#!anes( )o"t* so!es( pa!otid gland s1elling and
pain
ANA &C) o! R*e")atoid %acto!
&C): Lac' speci%icit$
Anti5SSA?SS2 &C): )a!'e!s
4!io! dx o% CT d/
2iops$: -ocal l$)p*oc$tic
sialadenitis &:>= )ono)"clea!
cells a!o"nd salia!$ gland
acini?d"cts)
-oc"s sco!e :1?<))
.
&V o%
%oci?gland"la! tiss"e SA)
In%ilt!ate o% #enign
l$)p*oepit*elial cells into
lac!i)al( pa!otid( salia!$ glands
CD<C )ostl$
Dest!"ction o% gland: -i#!osis(
!eplace)ent #$ %att$ tiss"e
DISEASE EPIDEMIOLOGY/
PATHOGENESIS
CLINICAL LAB PATHOLOGY
A#6i#is: S$))et!ical pol$a!t*!itis #"t no e!osions
A!t*!algia( pol$a!t*!itis( )$algia( )$ositis( We!ode!)a( p"!p"!a(
asc"litis( 4e!ip*e!al ne"!opat*$( c!anial ne"!opat*$( CNS
inole)ent( Rec"!!ent #!onc*itis( inte!stitial l"ng disease
R7EUMATOID
ART7RITIS
A"toi))"ne: T l$)p*oc$tes
T cells p!od"ced c$to'inesT cell
!ec!"it)ent c*!onic
in%la))ation#one( ca!tilage(
liga)ent and tendon
da)agee!osions( Point de%o!)it$
S$noi"): T$pe I0
TN-5
Mediates pat*ologic in%la))ation
Mediates Point dest!"ction
Mediates s$ste)ic(
ext!a5a!tic"la! s$)pto)s o%
in%la))ation
Reg"lates leels o% ad*esion
)olec"les !esponsi#le %o! le"'oc$te
)ig!ation
Assoc3 1?s*o!tened li%e span
Bila#eal7 sy++e#ical "!lya#6i#is o% Points: S1ollen( tende!
Lo15g!ade %ee!( 1t loss( ano!exia( %atig"e
Ext!a5a!tic"la! inole)ent:
L"ngs( s'in( s"#c"taneo"s tiss"e( *ea!t( *e)atologic s$ste)
Ra!el$: CNS( 'idne$s
Di%%e!entiate %!o) d!"g side e%%ectX
Spine: C15C. insta#ilit$
7ands: Ulna! deiation( s1an5nec' de%o!)it$ &a#oe DI4)(
#o"tonnie!e de%o!)it$ &#?t MC4 ADI4)( ca!pal t"nnel?,"$on cannal
s$nd!o)e( %lexo! tenos$noitis( extenso! tendon !"pt"!e
Ynee: S$noitis+ 2a'e!Ns c$sts
-eet: Coc'5"p de%o!)it$( talonaic"la! Point p!onation( ee!sion o%
%oot( an'le Point ta!sal t"nnel s$nd!o)e
C!ite!ia: < o% I
1)M!nin% s#i&&ness at least 1 *o"! %o! OF1's
.)S1elling o% O6 Points si)"ltaneo"s %o! OF 1's
6)S1elling o% 1!ist( MC4( 4I4 %o! OF 1's
<)S$))et!ical s1elling
>)7and WR c*anges: e!si!ns( P"xta5a!tic"la! de)ine!ali/ation
F)S"#c"t nod"les: extenso! s"!%ace( P"xta5a!tic"la!
I)A#no!)al se!") R*e")atoid %acto! o! anti5c$clic cit!"lli)ated
peptide A#
Co"!se:
13 4ol$c$clic: Most co))on
Re)ission?exace!#ation &156 c$cles)+c*!onic
.3Monoc$clic: 6>G
634!og!essie: 1>G5indolent onset( slo1l$ p!og!essie( non5
!e)itting( p!e)at"!e deat*
Tx: S"pp!ess in%la))ation
E pain( )aintain %xn( co!!ect speci%ic i)pai!)ents
H ESR
Ane)ia
T*!o)#oc$tosis
H Q2C
7$pe!Zglo#"line)ia
7$poco)ple)ente)ia
R8 &C): Co!!elates 1?see!e(
!e)itting d/
aCCP: Detecta#le $!s #e%o!e
clinical s$)pto)s( p!edictie o%
e!osieness
R- A aCC4 toget*e!: 7ig*
speci%icit$( lo1 sensitiit$
H Q2C in s$noial %l"id