Presentation SLE

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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