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8/19/2019 Presentation SLE
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8/19/2019 Presentation SLE
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Systemic lupus erythematosus in children
Josua Sitorus and Sri Wulandari
Supervisor:dr. Selvi Nafanti M.Ked(Ped),SpA(K)
Medical Facult !niversit o" Su#atera !tara$ %a&i Ada# Mali' eneral
%ospital
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Systemic Lupus Erythematosus (SLE)
Sste#ic lupus erte#atosus (S*+) is a co#ple, #ultisste#
autoi##une disease -ic results "ro# te interpla o"
environ#ental, or#onal and enetic "actors.
*upus can /e a #ild disease, a severe and li"e0treatenin illness,
or antin in /et-een
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Introduction
1e diversit o" clinical s#pto#s in S*+ is reat, and all oransste#s are vulnera/le
Widespread in2a##ation o" vessels and connective tissues
Presence o" antinuclear anti/odies
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Epidemiology
Pediatric S*+ represent 340567 o" all S*+
Prevalence: 58396$366,666 -orld-ide /ut as i as
56$366,666
;ncidence: 3836$366,666 -orld-idePopulation at iest ris':
Fe#ale:#ale ratio is approi#atel lac' (?@),
%ispanic and Native A#erican (58?), and
Asian (5) populations
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+tiolo
Gene
Environmen
t
Infection
Hormones
Etiology is Unknown
Antigen
Hormones (estrogen)
Infections
Toxins
Medications
Sun exposure
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• Rate of SLE concordance in monozygotic twins is 24!"#$ in
dizygotic twins is 2!#
• %&!%2 of SLE 'atients (ave %st) or 2nd)degree relatives
wit( SLE com'ared wit( *% in (ealt(y individ+als
• SLE 'atients may (ave family mem,ers wit( ot(er
a+toimm+ne diseases
Genetic Susceptibility—Clinical Studies
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+tiolo
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Patoenesis
From Bertsias GK Salmon !E Boumpas "#$ #herapeutic opportunities in systemic lupus erythematosus% state o& the art and prospect
&or the ne' decade$ nn heum "is *+,+-./%,.+01,,$
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+a#ples o" ;##une Bsreulationin *upus
2 -)cells
8 Be"ective selection$sinalin
8 Autoanti/od production
C .)cells
8 ;ncreased nu#/ers o" 13 and 15 cells and decreased nu#/ers o" 1res8 10cells are less suscepti/le to activation0induced cell deat
C /lasmacytoid dendritic cells
8 Produce lare a#ounts o" inter"eron
8 Plas#actoid dendritic cells: Sti#ulate activation andproli"eration o" autoreactive 10 and >0cells
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Patoenesis o" *upusD ;#portantEoncepts
2Autoi##unit is an altered i##une o#eostasis tat leads to
autoreactivit, i##unodefcienc, and #alinanc
C ;##une dsreulation leadin to autoreactivit and
autoanti/odies in S*+ occurs in dierent pases and li'el
represents te unto-ard eects o" environ#ental triers on teeneticall suscepti/le ost
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C Malar ras
C Biscoid ras
C Potosensitivit
C Gral ulcers
C Artritis
C Serositis
C lo#erulonepritis
C Neuroloic disorder:
SeiHures and$or pscosis
C %e#atoloic disorder:
;##une0#ediated
e#oltic ane#ia,
leu'openia,
C l#popenia,
C tro#/octopenia
C Antinuclear anti/odies
(ANA)
C ;##unoloic disorder:
anti0BNA anti/od, anti0
S#
anti/od, or
AEI (Ievised) Eriteria "orElassifcation
9$33
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Medical ;llustration Eoprit O 5635. Nucleus Medical Media. All ritsreserved
E0am'les of 1rgans Involved Signsand Sym'toms
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Synovitis 3alar
Ras(
1ral
+lcer
iscoid ras(
S+,ac+tec+taneo+s l+'+seryt(ematos+s
5asc+litis
L+'+s 'rof+nd+s 6acco+d7sart(ro'at(y
L+'+s on t(e 1+tside
Sste#ic *upus +rte#atosus Gvervie- Br. raciela Alarcn 1e !niversit o" Ala/a#a at >ir#ina#
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Serositi
s
/ericardial
e8+sion
C
Spherocytes
Glomer+lone'(ritis
9ere,ral infarct -rain atro'
Spherocytes
L+'+s on t(e Inside
Sste#ic *upus +rte#atosus Gvervie- Br. raciela Alarcn 1e !niversit o" Ala/a#a at >ir#ina#
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iagnosis andiagnostic .ests
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CAutoanti/odies aainst various co#ponents o" te cell nucleus
CPresent in #an autoi##une disorders as -ell as so#e ealt su/&ects
CSensitive (not specifc "or S*+)
:;:
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C>ecause o" lo- specifcit, ANA use"ulness increases i" te pretest
pro/a/ilit "or lupus is i ie, te patient as s#pto#s and sins tat
can /e attri/uted to S*+
C>ecause o" te i sensitivit o" te ANA, a patient -it neative ANA is
unli'el to ave lupus even -en er$is clinical presentation is suestive
o" lupus
:;:
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•;ormal s+,
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:nti,odies L+'+sS'eci?city
9linical :ssociations
:;: Low ;ons'eci?c
:nti)ds;: Hig( ;e'(ritis
:nti)Sm Hig( ;ons'eci?c:nti)R;/ Low :rt(ritis myositis l+ng
disease
:nti)SS: Low ry eyes@mo+t( s+,ac+tec+taneo+s l+'+seryt(ematos+s AS9LEBneonatal l+'+s'(otosensitivity
:nti)SS- Low Same as a,ove
:nti'(os'(oli'id
Intermediate 9lotting diat(esis
:+toanti,odiesin SLE
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/redictors of Care Ain some ,+t not all casesB ! ;ew evidence of com'lement cons+m'tion
! Rising anti)ds;: titers ! Increased ESR ! ;ew lym'(o'enia
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Polarticular diseases
!ndierentiated connective tissuedisease
lo#erulonepritis
Fever or spleno#eal$l#padenopat
Pul#onar8renal sndro#e
Ieu#atoid artritisStillQs disease
S&RrenQs sndro#e,Antipospolipid sndro#e,Fi/ro#alia -it positive ANA,
;diopatic tro#/octopenic purpura,Bru induced lupus
Post0in"ectious lo#erulonepritis(streptococcal, staplococcal)Me#/ranoproli"erativelo#erulonepriti;n"ectious diseases or l#po#a
oodpastureQs sndro#e, orantineutropilEtoplas#ic anti/od (ANEA) associatedvasculitis
i8erential iagnosis
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9(aracterized ,y
! :,r+'t onset of sym'toms
! Increased renal ne+rologic (ematologic and serosalinvolvement
! Ra'id accr+al of damage Airreversi,le organ inernats' S, >oivin JF, Josep *, et al. Artritis Ieu#. 56649:54460544.
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Goals of t(era'y ! Sto' and reverse ongoing organ inCammation
! /revent or limit irreversi,le end)organ damage
/otential to0icities of imm+nos+''ressive t(era'ies demandvigilant management
.(era'e+tic /rinci'les
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C Eorticosteroids C Eclopospa#ide C Metotreate
C Mcopenolate #o"etil C AHatioprine
C %droclorouine C >eli#u#a/
Current #herapy &or SLE
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• Imm+ne targeted t(era'y ! -)cell directed ! 9ytokine in(i,itors
! 9ostim+lation ,lockade ! /e'tide in(i,itors ! inase in(i,itors
! . reg+latory cells • Stem cell trans'lant
;ew .(era'e+tic Strategies
.argeted Imm+not(era'y
Tildiri#01oruner E, Bia#ond >. J Aller Elin ;##unol. 563335:?6?0?35.
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Current #herapy—Limitations
Imm+nos+''ressive dr+gs confer an
increased risk for ! Infection ! 9ancer ! Infertility
9ommon side e8ects of corticosteroids
! Infections ! 9+s(ingoid a''earance ! 3ood dist+r,ances ! 1steonecrosis
! ia,etes ! 1steo'orosis
! Hy'ertension ! Li'id a,normalities
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.(era'e+tic com,inations aimed atind+ction of remission maintenance
t(era'y and s+''ortive t(era'y
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9ase
/resentation
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IN, a 390ear0old irl presented to pediatric division, ada# #ali' ospital -it aco#plaint continue ce#oterap.
1e patient as /een enrolled in te division o" aller i##unolo Ada# Mali'
ospital -it te dianosis o" Sste#ic lupus erte#atosus.
%istor o" previous illness : Patients co#e frst in Septe#/er 5634 -it a istor o" &oint pain and s-ellin in te ands, 'nees, and an'les .pain -as "elt alread "or 3
-ee'. Joints pain ettin -orse in te #ornin, especiall on -a'in.
Ieddis ras eperienced / te patient in si#ultaneousl -it pain. 1e ras "elt
eat and itcin. 1e ras initiall patc reddis spots alone and increasinlspread on /ot sides o" te cee's. Ias -orsens -en eposed to sunlit.
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Patients eperienced air loss since 5 -ee's.
!rination a/nor#alit denied.
Eo#plaints o" pale and ello- /od denied.
Present seiHures denied.
Previous istor o" seiHures eperienced / patients until tere is a decrease
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%istor o" disease: land 1>E, Sste#ic lupus
erte#atosus%istor o" #edication :Metlprednisolone in&ection, EPA in&ection,
#etlprednisolone ta/let
%istor o" "a#il : None
%istor o" parentQs #edication : None
%istor o" prenanc: < #onts in te ae
5
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%ead:%air and scalp : alopesia
Face : ede#a (0), #alar ras (U), discoid ras (U)
+e : endo"tal#us (0), eo"tal#us (0), lit re2e (U$U),
isocoric pupil, palpe/ral con&unctiva pale (0$0),sclerai'eri' (0$0),
nor#al vision
+ars : /ot ear lo/e in nor#al #orpoloic, ear discare (0$0), in2a##ation
(0$0), i#purities (0$0), dirt (0$0),
Nose : septu# deviation (0), polp(0$0), i#purities (0$0) secret (0$0), nasal
canule (U), pin'is concae
Mout : canosis on lips (0), pin'is oral #ucose, pseudo#e#/rane (0),
detritus (0), secret (0), tonue #easure#ent nor#olosia, tonsil
#easure#ent 13
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;eck F -+llneck A)B tyroidmeas+rementA)B Lym'( node enlargement A)Bneck sti8ness A)B 65/ A)B
.(ora0 F
Ins'ection F -arrel c(est A)B /igeon c(est A)B D+nnel c(estA)B
Symmetrical f+siform retraction A)B /al'ation F stem fremit+s leftJrig(t normal condition wit(in ,ot(
l+ngsK
/erc+ssion F
L+ngs F sonor on ,ot( l+ngsK
Heart F U''er ,arrier F I9S III sinistra Rig(t ,arrier F I9S 5 L/S de0tra
Left ,arrier$ I9S 5 % cm medial L39S
:+sc+ltaionF
L+ngs F -reat(ing so+nd Fvesik+ler :dditional so+ndFronkiA)@)B w(eezing
A)@)BHeart F S%S2 AB S"A)B S4 A)B m+rm+r A)B
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V A/do#en :
;nspection : Si#etris, ascites (0)
Palpation : Soepel, liver unpalpa/le, 'idne unpalpa/le spleen unpalpa/le, tu#or
unpalpa/le
Percussion: Si"tin dullness (0), costoverte/ral pain (0),
Auscultation: Nor#operisalti', dou/le sound (0)
V +tre#ities : Pulse L6/p#, reular,adeuate p$v, "elt -ar#, EI1 ?X, pittin
oede#a (0$0), #uscle riidit (0)
V Anoenital : Fe#ale
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i8erential diagnosisF Systemic l+'+s
erit(ematos+s
r+g ind+ced l+'+s
R(e+matoid art(ritis
Di,romyalgia wit(
'ositive :;:
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Morking diagnosis F Systemic l+'+s
erit(ematos+s
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1est Iesult !nit Ie"erences
%e#olo/in 33,46 7 33.?039.3+rtrocte ?.4 36$##? 9.9609.9L
*eucocte 9,5 36?$##? .603.4
1ro#/octe 3L< 36?$##? 5309asopil 6.
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9ar,o(ydrate 3eta,olism
-lood Gl+cose N#K#& mg@dL * 2&&
Renal D+nction
Ure+m %&KN& mg@dL * #&
Ereatinine 6.94 mg@dL &K24)&K4%
Electrolyte
9alci+m A9aB NK> mE@L >K2)%%K&
;atri+m %4" mE@L %"#)%##
/otassi+m "K> mE@L "KO)#K#
9(loride %&> mE@L >O!%&O
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Liver
.otal -ilir+,in &K"% mg@dL *%
irect -ilir+,in &K%# mg@dL &)&K2
:lkali/osfataseA:L/B %44 U@L *%NP
:S.@SG1. %PN U@L *"2
:L.@SG/. P& U@L *"%
:l,+min 2KO g@dl "K2)4K#
Im+noserology
:;: .est %4> *2&
:nti ds);: NO2K& &)2&&
9R/ +antitatif *&KP mg@dL "KO)#K#
La, Res+lt onSe'tem,er 2&%#
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Q ;ormal diet %N&&kcal wit( P2mg 'rotein
Q In
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S 1 : /
Fever (0)
Eou (0)
=o#itin (0)
Malar Ias
(U)Alloplesia (U)
EontinuousEe#oterap
SLE
CQ ;ormal diet
%N&&kcal wit( P2mg
'rotein
CQ In
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S 1 : /
Fever (0)Eou (0)
=o#itin (0)
Malar Ias(U)
Alloplesia (U)
EontinuousEe#oterap
SLE
Q ;ormal diet %N&&kcal wit(
P2mg 'rotein
Q In
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S 1 : /
Fever (0)
Eou (0)
=o#itin (0)
Malar Ias(U)
Alloplesia (U)
Eontinuous
Ee#oterap
*a/ Iesults:
SG1$AS1: LL !$*
SP1$A*1 : L !$*
SLE
Q ;ormal diet %N&&kcal wit(
P2mg 'rotein
Q In
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isc+ssion
1%+GIT EAS+
+P;B+M;G*GT : Pediatric S*+ (pS*+) represents approi#atel
34056 7 o" all S*+ patients. ;t is #ore co##on in "e#ales tan in
#ales, -it a "e#ale to #ale ratio varin "ro# 5.?:3 to
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E*;N;EA* MAN;F+S1A1;GN $ E*AS;F;EA1;GN AEI :Malar ras
Biscoid ras
Potosensitivit
Gral ulcers
Artritis
Serositis
lo#erulonepritis
Neuroloic disorder: SeiHures and$or pscosis
%e#atoloic disorder:;##une0#ediated
e#olticane#ia, l eu'openia,
l#popenia,1ro#/octopenia Antinuclear anti/odies (ANA)
;##unoloic disorder:anti0BNA anti/od, anti0
S#anti/od, or antipospolipidanti/odies
Malar ras
Potosensitivit
Artritis,
A/nor#alit antinuclear anti/odies
ANA test Y ds0BNA
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1I+A1M+N1:
Eorticosteroids Eclopospa#ide
Metotreate Mcopenolate #o"etil
AHatioprine %droclorouine Iitui#a/
Eorticosteroid: Metlprednisolone
Eclopospa#ide
*A> B;ANGS1;E: Etopenias (ane#ia, tro#/octopenia, leu'openia)
+levated +SI,
EIP, ;##unolo/ulins
%poal/u#ine#ia
Proteinuria I>Es, casts in urine
Becreased creatinine clearance
*o- co#ple#ent levels (E?$ E9)
Autoanti/odies (ANA, AP*, Eoo#/s, anti0platelet A/,
reu#otoid "actor, etc.)
+levated +SI
AS1$A*1 : 394$35
Becreased cratinine
ANA test U
ds0BNA U
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Eonclusion:
IN, a 39 ears old irl, -it ? ' o" /od -eit and 344 c# o" /od
eit, ca#e to IS!P %a&i Ada# Mali' Medan on Januar, 33 563. %is #ain
co#plaint is to continue te ce#oterap. Patient -as reistered as
alleric and i##unoloic divisionQs patient in Ada# Mali' %ospital
dianosed -it Sste#ic *upus +rte#atosus. Se -as pri#aril
dianosed -it Sste#ic *upus +rte#atosus and treated -it ;n&.
Metlprednisolone 3666 # in 366cc NaEl 6.
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#han7 8ou
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