rheum dis in child 07

8/18/2019 Rheum Dis in Child 07

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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,


12/50#R(-R(S+


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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


15/50Rash


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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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rheum dis in child 07

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