Post on 07-Jul-2018
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Sumadiono
Department of Pediatric Fac. of Medicine Gadjah Mada University
Yogyakarta
RHEUMATIC
DISEASES
IN CHILDREN
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RheumaticDiseases
n !hi"dren
# group of diseases in chi"dren
Simi"ar manifestations
#cute$chronic in%ammation
in the Muscu"oske"eta"& 'ascu"ar& Ski
(he cause is unkno)n
(he precise diagnosis is di*cu"t
Dianosis+ ,y the criteria
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Treatment and Outcome
Growth & Development
Emotional and Social Efects
!-DR/0 )ith RheumaticDisorders
DIFFERS from ADULT
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Juvenile
Spondylo-
arthropathies
Juvenile
RheumatoidArthritis
CollagenVascular
!#SSF!#(10
22 344 rheumatic disorders inchi"dren
Three broad categories
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5 R #+ P o l y a r t i c
u l a r J R A
P a u c i a r t i c u l a r J R A S y s t e m i c J R A
!o""agen 'ascu"ar+ Sc"eroderma
Dermato myositis S/
6a)asaki Disease -enoch
Schon"einPurpura Rheumatic
Fever
JuvenileSpondylo-
Arthropathies:
Undifferentiated Psoriatic Arthritis Reactive Arthritis
JuvenileAnkylosingSpondylitis Bechets’ B!
!#SSF!#(10
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Polyarticular
JRA
Pauciarticular
JRA
SclerodermaDermato
myositis
Systemic
JRA
SLE
KawasakiDisease
Henoch
Schonlein
Purpura
Rheumatic
Fever
UndifferentiatedBechets’
Juvenile
Ankylosing
SpondylitisPsoriatic
ArthritisIBD
Reactive
Arthritis
CLASSIFICAI!"
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5U'/0/ R-/UM#(1D#R(-R(S
(JR!
JR is the most commonclass o" childhood rheumaticdisease
Disorders group with#
$hronic S%novitis
E'tra articular mani"estation
n ) *+ %ear
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5R#+
*,-+, $ases.*,,/,,,children
0emale 1 male
2ea3#0emale# *45 %6ale# 748 %
Earl% onset# +month
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imping Sti7ness of joints
on )a"king Re"uctance to
use a "im,
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#06Y1S0G+#06Y1S0G+
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9epatosplenomegali
0ever (intermitten!
2ericarditis
2leural efusion
Spond%litis
Er%tema palmaris
S:STE6$ 6;0ESTTO;S
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rheumatoidnodu"
vascu"itis
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@aborator%#@aborator%#
nemia @eucoc%tosis? neutrophil 11/
Thromboc%te
ESR $R2
;
R0 $5 & $A
gG serum
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Radiolog%Radiolog%
/ar"y+ no a,norma"ity
S)e""ing soft tissue
22 joint space
1steoporosis 0e) ,one formation
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DG;OSS
5R# is de8ned+ #rthritis in a chi"d 9$: 3; of age
n one or more joint
For si< )eeks or "onger
1ther diagnostic possi,i"ity has
,een e
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(hree su,types of 5R#+
=5uveni"e diophatic #rthritis$5# :
5R#>
3. Pauciarticu"ar 5R# =: 1"igoarticu"ar 5#>
?. Po"yarticu"ar 5R#+
Rh=@>+ chronic disease and Rh=A>+,etter
B. Systemic 5R#
/
P#U!#R(!U#R$1G1#R(!U#R 5R#
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P#U!#R(!U#R$1G1#R(!U#R 5R#
Relative "reBuenc% # A,4AC
E'traarticular "eature # >veitis#7C
;umber o" oint # ).F A 2ea3 onset age (%ear! # *45 %
0emale.6ale ratio # A#*
$ourse and prognosis# S%stemic disease absent 6aor cause o" morbidit% is
uveitis 2rognosis e'cellent
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2O@:RT$>@R JR2O@:RT$>@R JR
Relative "reBuenc% # A,4
AC
;umber o" oint # 1 C
2ea3 onset age (%ear! # *45? 18
0emale.6ale ratio # C#*
$ourse and prognosis#
S%stemic disease generall%mild
2ro nosis moderatel ood
E'traarticular
"eature# @ow grade
"ever >veitis# C Subcutaneus
nodule
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S:STE6$ JRS:STE6$ JR Relative "reBuenc% # *,
;umber o" oint # variable 2ea3 onset age # none 0emale.6ale ratio # *#*
E'traarticular "eature "ever @%mphadenopath%?
9epatomegal% Rash 2ericarditis
$ourse and prognosis#S%stemic & arthritis is chronic
2rognosis is variable
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S2O;D:@ORT9RO2T9ES 2revalence# *+/8.*,,?,,,
-S(1RY+ */ 6ale more o"ten
afected 7/ @ate onset age4ussuall%
1 *, %ears 5/ 0amil%# similar
disorder
!0!# SG0S+
*/ cute iritis 7/ 'ial spineinvolvement
5/ Sacroiliits A/ rthritis (large oint o"
lower limb!
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(he G1#S of (R/#(M/0( ofrheumatoid diseases in chi"dren+
$ontrol disease activit% 6a'imie normal growth and development 6inimie chronic disabilit% and de"ormit%
Remission o" the disorder
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(he (-/R#P/U(! (/#M comprises thefo""o)ing+
patient with parents . "amil% "amil% practitioner . pediatrician
local pediatric hospital
pediatric rheumatologist . special hospital orthopedist
ophthalmologist
ph%siotherapist ergotherapist
social wor3er
2s%chologist
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(R/#(M/0( of 5R#
Pauciarticu"ar 5R#+
;SD# spirin# Re%e S%ndrome
bupro"en# good? no re%e S%ndrome
;apro'en# Standard
;TRRT$>@R STEROD
t ti l ti
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ntra4articular nection# Triamcinolone 9e'atomide
@ab "or intra4artic Huid#cell.I$? culture? T (i"
needed! 0ollow up# *47 wee3s
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ntra4articular nection#
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TRET6E;T o" JR
Po"yarticu"ar 5R#+
;SD 6t' orall%
9%dro'%$hloroBuine
athioprine
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TRET6E;T o" JR
Sytemic 5R#+
;SD Steroid 6t' KG
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(reatment ofSpondy"oarthritis+
Steroid $%closporin
Other mmunosupresiveagents
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SYS(/M!UPUS
/RY(-/M#(1SUS
=S/>
S%stemic @upus Er%thematosus
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S%stemic @upus Er%thematosus
4 a challenging disease with a wide variet%o" presentations
4 man% s%stems can be afected separatel%or at the same time
4 occurs more commonl% in girls
4 considered the diferential diagnosis o"
man% s%mptoms and signs
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non organAspeci8c4
SYMP(1MS+
Fever Fatiue
Eeight "oss
-eadache!ognitive impairment
Psychosis& seiure
Rash
!hest painShortness of ,reathness#,domina" pain#rthtra"gia joint s)e""ing
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non organAspeci8c4
SG0S+#"opecia& rashMucous mem,rane u"cers
ymphadenopathyP"euritis& interstitii" pneum.Pu"monary hypertentionPeri$myo$endoAcarditis
-ypertension-epatosp"enomega"yPeriphera" 'asc. nsu7.Periphera" neuropathy
#rthritis
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Malar "#utterfly" rash
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Mucous mem,ane u"cers
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Discoid Lupus
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Vasculitis
agnos s# A "rom ** R
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agnos s# A "rom ** Rcriteria
*/ Eritema malar (utterH% rash! 7/ Discoid lupus 5/ 2hotosensitivit% A/ >lseration (mucocutaneus? oral? nasal!
C/ rthritis +/ ;ephritis? proteinuria1,?C g.da% L/ Ensephlophat%# convulsiion? ps%chosis M/ 2leuritis? pericarditis 8/ $%topenia *,/ b/ anti ds D; ? anti Sm? @E cell **/ ;
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(R/#(M/0( of S/+
Steroid
9%dro'%chloroBuine mmunosupressive#
$%closporin
KG
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2rognosis#
4 unpredictable disease and ma% be "atal
4 Nidne% disease is one o" the li"e
threatening complications o" S@E
4 nvolvement o" the blood# bleeding? palloror ris3 o" in"ection
4
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-SPH
: anaphy"actoid or a""ergicpurpura
a small vessels vasculitis
most "reBuent in children between 7 and M%ears
4 male#"emale 7#*
incidence ) *C %ears# 7*/L.*,, ,,,A4+ %ears # L,/5.*,, ,,,
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!"inica" manifestations+
4 palpable non4thromboc%topenic purpura4 cramp% abdominal pain
4 and arthralgia
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!"inica" manifestations+
4 palpable non4thromboc%topenic purpura4cramp% abdominal pain
4 and arthralgia
4 subcutaneous edema
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Treatment
Supportive
on"y occasiona" use of corticosteroids forthe gastrointestina" comp"ications
!omp"ication+ Rena" Fai"ure
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