juvenile rheumatoid arthritis.pptx

7/25/2019 Juvenile Rheumatoid Arthritis.pptx

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JUVENILE RHEUMATOID

ARTHRITIS

Pragna Vanapala

SGU SOM

September 4th, 2015


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Background n!ormat"on

#l$o kno%n a$ &u'en"le "d"opath"c arthr"t"$

Mo$t common rheumat"c d"$ea$e "n ch"ldren and one o! the more commonchron"c "llne$$e$ o! ch"ldhood

()#*(# + a group o! d"$order$ that all $hare cl"n"cal man"!e$tat"on$ o!arthr"t"$

Etiology and pathogenesislargel unkno%n, and genet"c component "$comple- $o d"$t"ngu"$h"ng b*% d"!!erent $ubtpe$ "$ d"!!"cult

Multiple classiication sche!as"a. /la$$"!"cat"on /r"ter"a o! the #mer"can /ollege o! )heumatolog #/). character"e$d"$ea$e "nto 3 on$et tpe$

1 Polarthr"t"$2 Ol"goarthr"t"$ * Pauc"art"cular

3 S$tem"c "$ea$e

b. nternat"onal 6eague o! #$$oc"at"on !or )heumatolog + u$e$ term (u'en"le idiopathicarthr"t"$ (#.

7 nclude$ abo'e three 8 P$or"at"c arthr"t"$, 9nthe$"t"$7related arthr"t"$, und"!!erent"ated


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/r"ter"a !or cla$$"!"cat"on o! (u'en"le )heumato"d #rthr"t"$

#ge at on$et :1; r

#rthr"t"$ $%ell"ng or e!!u$"on, or the pre$ence o! 2 or moreo! the !ollo%"ng $"gn$ l"m"tat"on o! range o! mot"on,tenderne$$ or pa"n on mot"on, "ncrea$ed heat. "n


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#/) '$ 6#) /la$$"!"cat"on


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#rthr"t"$

300,00 ch"ldren "n US ha'e arthr"t"$?100,000 %"th a !orm o! (#


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9t"olog o! ()#

@ot completel under$tood

2 component$ nece$$ar "mmunogenet"c $u$cept"b"l"t ande-ternal tr"gger

Mult"ple gene$ ma a!!ect d"$ea$e $u$cept"b"l"t A"$tocompat"bl"t comple- reg"on$ A6# cla$$ and allele$

a$$oc"ated %"th d"!!erent $ubtpe$ @on A6# loc"polmporph"$m$ "n gene$ that encode tumor necro$"$

!actor @C.7a, macrophage "nh"b"tor !actor, "nterleuk"n7;, and 671a @on genet"c tr"gger$ bacter"al and '"ral "n!ect"on$ par'o'"ru$ B1D,

rubella, 9p$te"n7Barr '"ru$., enhanced "mmune re$pon$e to bacter"alor mcobacter"al heat $hock prote"n$, abnormal reproduct"'e hormonele'el$, &o"nt trauma


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Pathogene$"$

()# "$ an auto"mmune d"$ea$e due to alterat"on$ "n both humoral and cell7med"ated "mmun"t

)ecru"tment o! lmphocte$ pr"mar"l tpe 1 helper cell$. relea$epro"n!ammator ctok"ne$ @C7a, 67;, 671. target $no'"al non7$el! ant"gen$

/omplement con$umpt"on, "mmune comple- !ormat"on, and b7cell act"'at"on al$o

promote n!lammat"on o! the $no'"um

"n!lammator $no'"t"$Inla!!ato#y syno$itis'"llou$ hpertroph, hperpla$"a %"th hperem"aand edema o! $no'"al t"$$ue

Aperpla$"a o! 'a$cular endothel"um and "$ "n!"ltrated b mononuclear, pla$macell$ %"th man lmphocte$

Per$"$tent "n!lammat"on can lead to pannus o#!ation abnormal laer o!

!"bro'a$cular t"$$ue or granulat"on t"$$ue !orm$ o'er the &o"nt., cart"lage ero$"on$,and de$truct"on o! underl"ng bone and other $tructure$ l"ke l"gament$ andtendon$ $urround"ng the &o"nt

@ote nher"tance o! $pec"!"c ctok"ne allele$ can al$o up regulate "n!lammator

med"ator$

$$tem"c on$et d"$ea$e or more $e'ere art"cular d"$ea$e


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/l"n"cal Man"!e$tat"on$ !or #ll pe$

#rthr"t"$ + mu$t be pre$ent !or d"agno$"$ o! an $ubtpe o! (# Deinition "ntra7art"cular $%ell"ng or pre$ence o! 2 or more $"gn$

l"m"tat"on "n range o! mot"on, tenderne$$ or pa"n on mot"on, and "ncrea$edheat or erthema

Morn"ng $t"!!ne$$ %"th l"mp or gell"ng $t"!!ne$$ a!ter re$t. a!ter"nact"'"t? a$$oc"ated %"th ea$ !at"gab"l"t and poor $leep>ual"t

n'ol'ed &o"nt$ $%ollen, %arm to touch, pa"n!ul on mo'ementor palpat"on %"th reduced range o! mot"on but u$uall not

erthematou$#rthr"t"$ "n larger &o"nt$ !"r$t accelerate$ l"near gro%th

a!!ected l"mb "$ longer, a$ "n!lammat"on per$"$t$prematureclo$ure o! gro%th plate $o $hortened bone$


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/l"n"cal Man"!e$tat"on$ cont

Bone m"neral metabol"$m and $keletal maturat"onare a!!ected "n e'er $ubtpe

Aa'e decrea$ed bone ma$$ o$teopen"a.

/tok"ne$ @C7a and 67;, ha'e deter"orat"ng e!!ect$on bone %*"n &o"nt and "n a-"al E append"cular bone$


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- F7ear7old bo %"th &u'en"le"d"opath"c arthr"t"$ 7

monart"cular "n'ol'ement o!the le!t knee

- n!lammator proce$$ep"ph$eal gro%th $t"mulat"on"n"t"al local bono'ergro%th and "ncrea$ed

bone lenghth - 9'entuall, premature

ep"ph$eal clo$ure$horten"ng o! the "n'ol'edbone

/l"n"cal Man"!e$tat"on$


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1 Ol"goarthr"t"$

Epide!iology Pauc"art"cular (#* Ol"goarthr"t"$mo$t common$ubtpe 507;0., more g"rl$ than bo$ are a!!ected 31., peak age o!on$et 274r$

=4 &o"nt$ %*"n !"r$t ;month$ o! d"$ea$e on$et, o!ten onl $"ngle &o"nt "$

"n'ol'ed#!!ect$ la#ge %oints o lo&e# e't#e!ities + knee$ and ankle$

! d"$ea$e ne'er de'elop$ "n more than 4 &o"nt$pe#sistentoligoa#ticula# JIA

! d"$ea$e "n'ol'e$ more than 4 &o"nt$ o'er t"mee'tended

oligoa#ticula# JIA %or$e progno$"$.Pre$ence o! po$"t"'e #@# te$t"ncrea$ed r"$k o! a$mptomat"c

anter"or u'e"t"$

! h"p "$ "n'ol'ed$ugge$t a $pondloarthropath, ne'er a $"gn o!ol"goarthr"t"$HHH


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Ol"goart"cular (u'en"le "d"opath"c arthr"t"$%"th $%ell"ng and !le-"on contracture o!r"ght knee

Ol"goarthr"t"$ contI


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2 Polarthr"t"$* Polart"cular "$ea$e

Epide!iology" Polart"cular 30735, more g"rl$ than bo$a!!ected 51. #ge o! on$et ha$ a b"modal d"$tr"but"on 274r,and 10714r$

n!lammat"on o! () %oints "n both upper and lo%er e-trem"t"e$

! rheumato"d !actor "$ pre$ent

$"m"lar to $mmetr"cpre$entat"on o! adult rheumato"d arthr"t"$

R* positi$e "nd"'"dual$ 7J #heu!atoid nodules on e-ten$or$ur!ace$ o! elbo%$ and o'er #ch"lle$ tendon$more $e'erecour$e

Mic#ognathiaunder$"ed &a%.re!lect$ chron"c M( d"$ea$e

+e#$ical spine in$ol$e!entdecrea$ed neck e-ten$"on,r"$k o! atlantoa-"al $ublu-at"on, and neurolog"c $mptom$

Hip diseasedecrea$ed and pa"n!ul range o! mot"on on e-am


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,olya#ticula# %u$enile a#th#itis"- Progre$$"on o! &o"nt de$truct"on

lo$$ o! art"cular cart"lage,de$truct"'e change$ "n PP$,P$, and metacarophalangeal&o"nt$,

- e$truct"on and !u$"on o! %r"$tbone$

)heumato"d nodule$ "n ach"lle$tendon e-clu$"'el $een "n )Cpo$"t"'e ch"ldren.

Polarthr"t"$* Polart"cular "$ea$e contI


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Polarthr"t"$* Polart"cular "$ea$e contI


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3 S$tem"c on$et d"$ea$e

Epide!iology" S$tem"c d"$ea$e 10720, no $e- predom"nance,occur$ throughout ch"ldhood %*out a peak

A#th#itis

Ce'er $p"k"ng temperature$ to < 30/ da"l or t%"ce da"l !or at lea$t2%k$ %"th rap"d return to normal or $ubnormal temperature$ O!ten pre$ent "n e'en"ng !re>uentl accompan"ed b !a"nt, erthematou$, macular ra$h

he $almon colored le$"on$ are l"near or c"rcular and d"$tr"buted

o'er the trunk and pro-"mal e-trem"t"e$ /la$$"c ra$h "$ nonprur"t"c and m"grator %"th le$"on la$t"ng :1hr

Visce#al in$ol$e!ent" hepato$plenomegal, lmphadenopath,$ero$"t"$ per"card"t"$.


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3 S$tem"c on$et d"$ea$e contI

Koebner phenomenon + cutaneou$hper$en$"t"'"t $k"n le$"on$. that appear at $"te o!$uper!"c"al trauma? heat !rom %arm bath can al$o

e'oke ra$hSome ch"ldren at the beg"nn"ng onl $ho% $$tem"c

!eature$, but de!"n"t"'e d"agno$"$ re>u"re$ pre$enceo! arthr"t"$

#rthr"t"$ can a!!ect an number o! &o"nt$, but "$u$uall polart"cular and al$o "n'ol'e$ h"p, cer'"cal$p"ne, and M(


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S$tem"c on$et (# $almoncolored le$"on$ on trunkand e-trem"t"e$

-oe.ne# pheno!enon"

l"near maculopapular le$"on$that %ere pro'oked b mak"ng$cratch mark$

S$tem"c on$et d"$ea$e contI


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S$tem"c on$et (# + Macrophage #ct"'at"on Sndrome

/ac0g#ound" # rare, but !atal compl"cat"on o! So(# that can occur at ant"me dur"ng cour$e o! d"$ea$e

#l$o kno%n a$ $econdar hemophagoct"c $ndrome or hemophagoct"clmphoh"$t"octo$"$ A6A.

Aemophagocto$"$ phagocto$"$ b h"$t"octe$ o! erthrocte$, leukocte$, platelet$, andthe"r precur$or "n bone marro% and other t"$$ue$

Etiology and ,athophysiology" r"gger l"ke a '"ral "n!ect"on or med"cat"on uncontrolled act"'at"on and prol"!erat"on o! macrophage$, lmphocte$, and an "ncrea$e"n c"rculat"ng ctok"ne$

,#esentation

#cute on$et o! $e'ere anem"a %"th thromboctopen"a or leukopen"a, h"gh $p"k"ng !e'er$,lmphadenopath, and hepato$plenomegal

#l$o ha'e purpura, muco$al bleed"ng, ele'ated !"br"n $pl"t product$, prolonged P andP, ecrea$e "n 9S) due to lo% !"br"nogen le'el$ and hepat"c d$!unct"on

Diagnosis cl"n"cal cr"ter"a, con!"rmed %"thbone marro% b"op$ $ho%"nghemophagocto$"$

T#eat!ent A"gh do$e V methlpredn"$olone, cclo$por"ne, or anak"nra


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Macrophage #ct"'at"ng S$tem

,RELIMINAR1 DIA2NOSTI+ 2UIDELINES *OR MA+RO,HA2E A+TIVATION S1STEM3MAS4 +OM,LI+ATIN2 S1STEMI+ JUVENILE IDIO,ATHI+ ARTHRITIS 3JIA4

LA/ORATOR1 +RITERIA

1 ecrea$ed platelet count L2;2 10D*6.2 9le'at"on$ o! a$partate am"notran$!era$e J5D U*6.

3 ecrea$ed %h"te blood cell count L40 10D*6.

4 Apo!"br"nogenem"a L25 g*6.

+LINI+AL +RITERIA

1 /entral ner'ou$ $$tem d$!unct"on "rr"tab"l"t, d"$or"entat"on, letharg, headache, $e"ure$, coma.

2 Aemorrhage$ purpura, ea$ bru"$"ng, muco$al bleed"ng.

3 Aepatomegal edge o! l"'er N3 cm belo% the co$tal arch.

HISTO,ATHOLO2I+ +RITERION

9'"dence o! macrophage hemophagocto$"$ "n the bone marro% a$p"rate

DIA2NOSTI+ RULE

he d"agno$"$ o! M#S re>u"re$ the pre$ence o! an 2 or more laborator cr"ter"a or o! an 2 or 3 ormore cl"n"cal and*or laborator cr"ter"a # bone marro% a$p"rate !or the demon$trat"on o!hemophagocto$"$ ma be re>u"red onl "n doubt!ul ca$e$


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6ab C"nd"ng$

9le'atedANA"n 4075 o! ch"ldren %* ol"goart"cularor polart"cular (# but rare "n So(# #@# $eropo$"t"'"t + a$$oc"ated %* r"$k o! chron"c u'e"t"$

5710 %* polart"cular (# are $eropo$"t"'e !or )C

#nt"7ccl"c c"trull"nated pept"de //P.marker !ormore aggre$$"'e d"$ea$e

/h"ldren %* So(# ele'at"on "n "n!lammator

marker$, B/, and platelet count$Ab le'el$ + F710g*dl %"th $"m"lar"t"e$ to anem"a o!chron"c d"$ea$e

9S) "$ u$uall h"gh e-cept "n M#S, !err"t"n "$ u$uall

ele'ated and can be "ncrea$ed "n M#S


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6ab C"nd"ng$ contI

)ad"olog"c change$ o! arthr"t"$ So!t t"$$ue $%ell"ng, per"art"cular o$teoporo$"$, per"o$teal

ne% bone appo$"t"on around a!!ected &o"nt$

Subchondral ero$"on, lo$$ o! cart"lage, bon de$truct"on,!u$"on

/hange$ "n cer'"cal $p"ne at /27/3 can progre$$ toatlantoa-"al $ublu-at"on

M) can detect change$ "n earl $tage$ o! d"$ea$e


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reatment

Goal$ o! treatment d"$ea$e rem"$$"on, pre'ent &o"nt damage,encourage normal gro%th and de'elopment

Pauc"art"cular d"$ea$e /an re$pond to@S#$ alone %"th "mpro'ement "n "n!lammat"on

and pa"n ho$e %* no re$pon$e a!ter 47;%k o! @S#$ or %* !unct"onal

l"m"tat"on$ &o"nt contracture or leg length d"$crepanc."ntra7art"cular cort"co$tero"d$ l"ke ra"mc"nolone he-aceton"de

"$ea$e mod"!"ng ant"rheumat"c drug$ M#)$.!or tho$e %* nore$pon$e to abo'e drug$ +Methotre-ate "$ lea$t to-"c

! $t"ll no re$pon$e to methotre-ateb"olog"c med$ that "nh"b"t@C7a and 671 @C7a antagon"$t$ etanercept, adal"mumab,"n!l"-"mab. are !or ch"ldren %* poor re$pon$e to Methotre-ate,poor progno$t"c !actor$ or %* $e'ere d"$ea$e


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reatment /ont"nuedI.

S$tem"c Stero"d$ nd"cat"on$ Se'ere "n!lammat"on S$tem"c "llne$$

Br"dge therap

/ontrol o! u'e"t"$ Stero"d$ $"de e!!ect$ /u$h"ng $ndrome, gro%th retardato"n,

o$teopen"a

Management o! (# ha$ to "nclude pe#iodic slit5la!popthalmolog"c e-am"nat"on$ to mon"tor !or a$mptomat"cu'e"t"$

Ophthalmolog !ollo% up, ph$"cal therap, occupat"onaltherap, d"etar e'aluat"on and coun$el"ng to make $ure there "$

ade>uate calc"um, '"tam"n , prote"n, and calor"c "ntake


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O'er'"e% o! ()#*(#


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O'er'"e% o! ()#*(# contI


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"!!erent"al "agno$"$

A#th#itis"Man"!e$tat"on !or man mult"$$tem rheumat"c d"$ea$e$ l"ke SLE6 %u$enile

de#!ato!yositis6 sac#oidosis6 $asculitic synd#o!esScle#ode#!a+ l"m"ted range o! mot"on due to $clerot"c $k"n o'er &o"nt can be con!u$ed%"th chron"c "n!lammator arthr"t"$Acute #heu!atic e$e# &o"nt pa"n E tenderne$$, !e'er, E m"grator polarthr"t"$n!ect"on$ a$$oc"ated %* arthr"t"$ pa#$o$i#us /786 #u.ella6 E/V6 Hep/6 HIV6 ente#ic

inectionsLy!e disease + ch"ldren %"th ol"goarthr"t"$ l"'"ng "n or '"$"t"ng endem"c area$-ingella 0ingae + monoart"cular arthr"t"$ unre$pon$"'e to "n!lammator t-Septic a#th#itis" acute on$et o! !e'er, pa"n!ul erthematou$, hot &o"nt$

Spondyloa#th#opathy" tenderne$$ o'er "n$ert"on o! l"gament$, tendon$, and lo%ere-trem"t"e$ "n a boInla!!ato#y .o&el disease" can man"!e$t a$ ol"goarthr"t"$, a!!ect &o"nt$ "n lo%er

e-trem"t"e$, G $mptom$, 9S) ele'at"on, m"croct"c anem"aLeu0e!ia o# neu#o.lasto!a can ha'e &o"nt or bone pa"n due to mal"gnant "n!"ltrat"on

o! bone, $no'"um, or bone marro% U$uall no bone pa"n on palpat"on A"gh 9S) %*leukopen"a and lo% normal platelet count clue to underl"ng leukem"a.


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Progno$"$

50 o! (# pat"ent$ ha'e act"'e d"$ea$e that per$"$t$ "nto earladulthood %* $e'ere l"m"tat"on$ to ph$"cal !unct"on

Per$"$tent ol"goart"cular d"$ea$e7 Good progno$"$ %* ma&or"t undergo"ng rem"$$"on

G"rl$ %* #@# po$"t"'e and on$et o! arthr"t"$ earl"er than ;r$ r"$k !or chron"c

u'e"t"$ %h"ch can cau$e po$ter"or $nech"ae, cataract$, and band keratopath lead"ngto bl"ndne$$

Polart"cular (# Aa$ prolonged cour$e o! act"'e &o"nt "n!lammat"on and re>u"re earl and aggre$$"'e

therap Se'ere and per$"$tent d"$ea$e "! oung age at on$et, )C $eropo$"t"'"t, rheumato"d

nodule$, ant"7//P ant"bod"e$, h"gh number o! a!!ected &o"nt$

S$tem"c (# Poorer progno$"$ "n ch"ldren %* "ncrea$ed "n!lammator marker$ !or J;mo, !e'er

la$t"ng J3mo, polart"cular d"$tr"but"on o! arthr"t"$


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)e!erence$

@el$on te-tbook o! Ped"atr"c$


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THE END

juvenile rheumatoid arthritis.pptx

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