The Management of Systemic Lupus Erythematosus

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    THE MANAGEMENT OF SYSTEMIC

    LUPUS ERYTHEMATOSUS

    Mulia

    September 2011

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    Itr!"u#ti!

    $ SLE i% a #&r!i# i'ammat!r(aut!immue "i%ea%e !) u*!+eti!l!,(- +it& .ariable #lii#almai)e%tati!- #!ur%e a" pr!,!%i%/

    $ It i% #&ara#terie" b( peri!"% !)remi%%i!% a" peri!"% !)ea#erbati!%- +&i#& ma( i.!l.ea( !r,a !r %(%tem i .ari!u%

    #!mbiati!%/ri#a C!lle,e !) R&eumat!l!,( A" H!# C!mmittee ! SLE ,ui"elie%/ Art&riti% R&eum 1342567189

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    $ I t&e USA- t&e pre.ale#e !) SLE i%1 7 1-000

    $ At RSCM- 1/4=!) t!tal !utpatiet%i t&e Clii# !) R&eumat!l!,( i 2002+a% SLE/

    $ Female 7 male > ;14 7 1

    $ T&e ,reat ma?!rit( !) t&e%e patiet%are +!me i t&eir #&il"beari,(ear%/

    eri#a C!lle,e !) R&eumat!l!,( A" H!# C!mmittee ! SLE ,ui"elie%/ Art&riti% R&eum 134256718a Reumat!l!,i I"!e%ia/ Pa"ua "ia,!%i% "a pe,el!laa SLE- September 2004/

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    $ Sur.i.al rate% are @90= at 10 (ear%a)ter "ia,!%i% a" @

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    Clinical Features

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    $ #riteria 5ANA;p!%iti.e6 alm!%t %urel( SLE/

    $ #riteria 5ANA;e,ati.e6 .er( l!+pr!babilit(/

    $ Ol( ANA;p!%iti.e uli*el( SLE/

    Specifcity Sensitivity

    4D11 #riteria @:= 9:=

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    Conditions that may beconfused with SLE

    $ U"ieretiate" #!e#te" ti%%ue"i%ea%e

    $ S?!,re% %("r!me$ Atip&!%p&!lipi" atib!"( %("r!me

    $ Fibr!m(al,ia +it& ANA 56

    $ I"i!pat&i# t&r!mb!#(t!peia purpura$ ru,;i"u#e% lupu%

    $ Earl( r&eumat!i" art&riti%

    $ a%#uliti%

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    Characteristics of Mild SLE

    S Lif th t i

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    Severe or Life-threateningSLE

    S Lif th t i

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    Severe or Life-threateningSLE

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    Assessment ofDisease Activity and Severity

    $ I% imp!rtat )!r t&e e%tabli%&met !)

    a appr!priate treatmet pr!,ram/

    $ I% ee"e" t! #l!%el( )!ll!+;up t&e

    a#ti.it( !) "i%ea%e/

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    Disease Activity Indees

    $ SLEAI5SLE i%ea%e A#ti.it( I"e 6

    $ MEJ;SLEAI5T&e Mei#a SLE i%ea%e

    A#ti.it( I"e6$ SLAM 5SLE A#ti.it( Mea%ure6

    $ BILAG I"e 5T&e Briti%& I%le% Lupu%

    A%%e%%met Gr!up6$ LAM; 96

    $ Real impairmet 5%#!re > 46

    $ Hem!li%i% D tr!mb!%it!peia 5%#!re > 6

    $ Mi!%iti% 5%#!re > 6

    $ Art&riti% 5%#!re > 26$ Mu#!#utae!u% impairmet 5%#!re > 26

    $ Ser!%iti% 5%#!re > 26

    $ Fe.er K 9C D )atiue 5%#!re > 16

    $ Leu#!p&eia D L(m)!p&eia 5%#!re > 16

    S#!re7 K12 > %e.ere "i%ea%e a#ti.it(

    12 > mil" t! m!"erate "i%ea%e a#ti.it(

    > .er( mil" !r ! "i%ea%e a#ti.it(

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    "he Aims of Management

    Geeral

    Spe#i#

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    "he #illar of "reatment

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    "he "reatment of Mild SLE

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    "he "reatment of Mild SLE

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    "reatment of Serious$ Life-threatening$

    or %rgan-threatening SLE

    $ T&e pillar !) treatmet i% %imilar t!mil" SLE- e#ept t&e p&arma#!l!,i#altreatmet/

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    Corticosteroids

    $ Pre"i%!e : ; 0 m, "ail( are ee#ti.ei treatmet !) mil";t!;m!"erate SLE-i#lu"i, #utae!u% "i%ea%e- art&riti%-a" %er!%iti%/

    $ M!re %e.ere !r,a i.!l.emet 5ep&riti%-peum!iti%- &emat!l!,i# ab!rmalitie%- CNS "i%ea%e-

    a" %(%temi# .a%#uliti%6reuire &i,& "!%a,e%!) pre"i%!e 1 ; 2 m,D*,D"a(/

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    Corticosteroids

    $ Li)e;t&reatei, SLE I pul%e MP51 ,6 )!r "a(%/

    $ Ster!i" #a a#t a% bri",i, t&erap()!r t&e %l!+er;a#ti,

    immu!m!"ulat!r( a,et%/

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    $ T&e "!%e #a t&e be tapere"+&et&e immu!m!"ulat!r be,i% t! ta*e

    ee#t/

    $ O#e t&e "i%ea%e a#ti.it( i% u"er#!tr!l- tapere" t! !e !r miimal"ail( 5pre"i%!e : m,D"a(6 !ralterate;"a( "!%i, )!r maitea#et&erap(/

    Corticosteroids

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    A&athio'rine

    $ A purie aal!,/

    $ Aat&i!prie 522/: m,D*,D"a(6 i% a%a alterati.e maitea#e t&erap(t! CY i patiet% +it& lupu% ep&riti%

    a" !t&er !r,a;t&reatei,mai)e%tati!%/

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    C + CY C

    C!rti#!%ter!i" itermittet CY

    5i. b!lu% re,ime% !) 0/:1,Dm2 BSA6

    Ha" %i,i#atl(better real %ur.i.al

    C!rti#!%ter!i" al!e

    /

    Cyclo'hos'hamide

    Au%ti H- et al/ N E,l Me" 19

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    $ T&e tra"iti!al CY re,ime7

    < t! 8 m!t&l( pul%e !) CY al!e !r +it&

    pul%e MP i t&e i"u#ti!- a" t&e uarterl(pul%e CY )!r 2 (ear%/

    $ I i)u%i! !) me%a ri,!r!u%&("rati! t! pre.et &em!rr&a,i#

    #(%titi% a" bla""er #a#er )r!ma#r!lei- a t!i# metab!lite !) CY/

    $ Si"e ee#t%7 i)ertilit("ue t!

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    $ RCT MMF appeare" t! be a% ee#ti.e a%i. CY i i"u#i, %&!rt;term remi%%i! !)lupu% ep&riti% +it& a better %a)et( pr!le/

    $ T&e r!le !) MMF i impr!.i, l!,term!ut#!me% !) lupu% ep&riti% remai%

    u*!+/

    $ T&e "!%i, ra,e )r!m :00m, t! 1:00m, t+i#e "ail(/

    Giler E- et al/ N E,l Me" 200:3:7221;2229/

    Myco'henolate Mofetil

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    %ther Drugs and Modalities

    $ Le'u!mi"e

    $ e&("r!epia"r!%ter!e 5HEA6

    $ T&ali"!mi"e$ Itra.e!u% immu!,l!buli 5IIG6

    $ Pla%ma e#&a,e !r pla%map&ere%i%

    $ Aut!l!,!u% Stem CellTra%platati!

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    SLE and #regnancy

    $ Sterilit( a" )ertilit( )ate% )!r +!me+it& SLE are #!mparable t! #!tr!l,r!up% +it&!ut "i%ea%e/

    $ T&e i#i"e#e !) 'are "uri,pre,a#( !r a)ter "eli.er( i#rea%eab!ut

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    1/ Pre,a#( !ut#!me i% !ptimal +&e"i%ea%e i% i #!mplete #lii#al remi%%i!)!r < ; 12 m!t&%/

    2/ C!rti#!%ter!i" %&!ul" be ,i.e a% l!+"!%e a% p!%%ible !) le%% t&a 20 m, "ail(/

    / Aat&i!prie#a be u%e" "uri,pre,a#(/

    &ar P- S!*!l R/ Cli Me" a" Re%ear#& 200

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    SLE and A#S

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    1/ Elimiate t&e Atip&!%p&!lipi" atib!"ie%b( u%i, Immu!%uppre%i.e "ru,%/

    2/ Impr!.e t&e ri%* )a#t!r% %u#& a% #ea%e !)#i,arette/

    / P&arma#!l!,(7 L!+ "!%e 590 m,6 "ail( !) A%piri/

    Ati#!a,ulat5UFH- LMQH- !r Qar)ari6

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    Lu'us (e'hritis

    $ Hi%t!pat&!l!,( )eature i% ee"e" t! #!rmt&e iitial treatmet #!rti#!%ter!i" al!eversus immu!%uppre%i.eD#(t!t!i# "ru,%/

    $ Salt re%tri#ti! i) &(perte%i! D e"ema/

    Pr!tei re%tri#ti! +&e real )u#ti!impairmet !) 40=/

    L!,;term Cal#ium %upplemet )!r t&!%e+&! ! #!rti#!%ter!i" K8/:m, "ail(/

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    Lu'us (e'hritis

    $ U%e "iureti# a% ee"e"/

    $ A.!i" NSAI%/

    $ M!it!ri, real )u#ti!7 urial(%i%-#reatiie- albumi- C- ati;"%NA- 24&!ur uriar( pr!tei- CrCl 1;2 +ee*l(/

    $ A,,re%%i.e treatmet !) &(perte%i!/

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    Lu'us Cerebral

    $ Hi,& m!rbi"it( a" m!rtalit( rate/

    $ T&e Ameri#a C!lle,e !)

    R&eumat!l!,(7 Mil" %(mpt!m%7 &ea"a#&e 5i#lu"i,

    mi,raie6- rea#ti.e p%(#&iatr( "i%!r"er-

    #!,iti.e "(%)u#ti!- aiet( "i%!r"er/ Se.ere %(mpt!m%7 a%epti# mei,iti%-

    )!#al "e#it eur!l!,( 5"ue t! C !r"em(eliati! %("r!me6- #&!rea-

    m(el!pat&(- %eiure- a#ute #!)u%i!al

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    $ T&e "ia,!%i% i% "i#ult- a% t&ere are

    ! %pe#i# lab!rat!r( te%t%/

    $ I %e.ere )!rm 5i#lu"i, .a%#uliti%67 i.

    pul%e %ter!i" MP 1 , "ail( )!r "a(%/

    $ I) ! re%p!%e I CY 0/8:

    1/00,Dm2 ;< +ee*l(/

    $ Clii#al %table pre"i%!e 1m,D*,D"a( i "i.i"e" "!%e/

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    M!it!ri, SLE

    $ Li)el!, m!it!ri, t! "ete#t'are% earl( a" i%titute pr!mpt-appr!priate t&erap(/

    $ It i% imp!rtat t! re"u#e m!"iableC ri%* )a#t!r% i#lu"i, t!ba##!

    u%e- !be%it(- %e"etar( li)e%t(le-"(%lipi"emia- a" &(perte%i!/

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    C!mplemet C- C4Titer !) ati;"%NA

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    C!mplemet C- C4Titer !) ati;"%NA

    T&e )reue#( !) t&e%e e.aluati!+ill "epe" !7/ T&e a#ti.it(- %e.erit(- a" etett&e SLE/

    / T&e re%p!%e t! treatmet// T&e t(pe !) treatmet

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    $ A "e#rea%e i %erum C-C4

    $ A i#rea%e i ati;"%NA

    $ A ri%e i ESR

    $ A "e#rea%e iHaem!,l!bi !r Leu*!#(te!r Platelet #!ut%

    $ A ri%e i CP le.el$ T&e appeara#e !)mi#r!%#!pi# &aematuria !rpr!teiuria

    Ma( pre#e"e a#lii#al "i%ea%e

    'are/

    M!"i#ati! !)treatmet

    Si,i#atl(re"u#e t&e

    #&a#e !) 'are

    C&a,e% i t&e#ertai lab!rat!r(

    re%ult%/

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    THE

    TEA

    M

    APP

    RO

    ACH

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    Summary

    $ SLE i% a #!mple "i%!r"er +it& .ariablepre%etati!%- #!ur%e- a" pr!,!%i%/

    $ T&e pillar maa,emet !) SLE #!%i%t% !)e"u#ati!D#!u%eli,- eer#i%eDre&abilitati!pr!,ram- a" p&arma#!l!,i#al treatmet/

    $ Li)el!, m!it!ri, i% reuire" )!r m!%tpatiet%/

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    Summary

    $ It %&!ul" be maa,e" b( a teamappr!a#&/

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    THAN YOU

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    S l i Ab liti i

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    Serologic Abnormalities inSLE

    lippel H- ieppe PA- e"it!r/ R&eumat!l!,(/ L!"!7 M!%b(319/

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    "he #illar of "reatment

    E"u#ati! D #!u%eli,-

    Eer#i%e D Re&abilitati! pr!,ram-

    P&arma#!l!,i#al treatmet7 NSAI%-

    Atimalarial "ru,%-

    Glu#!#!rti#!i"%- Immu!%uppre%i.eD#(t!t!i# "ru,%/

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    Myco'henolate Mofetil

    MMF i% a ia#ti.e pr!"ru, !)m(#!p&e!li# a#i" 5MPA6- +&i#&i&ibit% i!%ie

    m!!p&!%p&ate "e&("r!,ea%e-l(mp&!#(te pr!li)erati!-

    a" b!t& T; a" B;#ell )u#ti!/

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    F * D * b i

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    Fa*t!r pe#etu%De*%a%erba%i

    SLESLE

    Obat :Obat :

    KeguguranKeguguran

    KehamilanKehamilanTindakanpembedahan

    Tindakanpembedahan

    n!eksin!eksi

    Sinar "#$%&'()'' nm*

    Sinar "#$%&'()'' nm*

    r,cainami

    d-idrala.in/etild,paC01 2-

    r,cainami

    d-idrala.in/etild,paC01 2-

    #atients with )nown renal

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    #atients with )nown renaldisease

    $ A urial(%i%-

    $ 24;&!ur urie pr!tei-

    $ Creatiie %erum- Cr#l

    $ C!mplete bl!!"#!ut-

    $ Lipi" pr!le-

    $ Cal#ium- P&!%p&ate-$ S!"ium a" P!ta%ium/

    1D12at t&e !%et

    !) ep&riti%

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    #atients with )nown renal

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    #atients with )nown renaldisease

    $ A urial(%i%-

    $ 24;&!ur urie pr!tei-

    $ Creatiie %erum-

    $ C!mplete bl!!"#!ut-

    $ Lipi" pr!le-

    $ Cal#ium- P&!%p&ate-$ S!"ium a"

    P!ta%ium/

    1D12at t&e !%et

    !) ep&riti%

    M!re !)te

    I) t&e #!"iti!i% u%table