laporan sk4 klp 13

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TUTORIAL REPORT GROUP 13

(BLOK HEMATOIMUNOLOGY)

4th Module

Compiled By :

1. Analia 1318011009

2. Arli Suryawinata 1318011023

3. Ayu Wulan Sari 1318011029

4. Eka Enda !e"tari 13180110#2

$. %adel & 'krom 13180110#4

#. %adia Eryuda 13180110#$(. )leny" *ulanda 13180110(#

8. +ommana ,ulia 131801114$

9. -ara Aulianoa 13180111#2

10.-riola %itria 13180111(0

11./itoria awarima 13180111(4

MEDICAL FACULTY OF LAMPUNG UNIVERSITY

2015

Scenario o !"# Mo$%&e

1



%eelin iy and Blurin Eye" A5ter 'n6etion

&r". Sara 7 40 year" old ome to te dotor omplainin aout er 6oint

di"om5ort. Se 5eel" it eery mornin in er knee" and and". er knee i"

"wellin and "e 5eel ae in er arm. A5ter 5ollowin te i"torial di"ea"e"7 &r".

Sara "aid o5ten ad ou7 5lu7 and "oretroat. Beau"e "e" already ored 5or

on"umin dru"7 Sara want" to e in6eted y analeti. A5ter te dotor a"k"

aout i"tory o5 dru alleri and "e a"nt ae it7 Sara n in6eted wit

analeti. %ew time" later7 Sara 5eel" diy7 lurrin eye" and ard to reat.

Wat i" atually appened to Sara

STEP 1

2



STEP 2

3



1. ow ariou" omplaint" tat may our

2. Wat te diano"i" o5 te"e "enario

3. Wat kind te in6etion o5 dru" in te ody o5 ny. "ara tat makin er

5eel ored

4. A5ter te in6etion o5 te"e dru" 7 wat "ould dotor do

STEP 3

4



1. ; 'n5lammation Artriti" +eumatoid on oin and "pread to "truture"

6oint" and "urroundin area" inludin 6oint artilae and 5irou" 6ointap"ule.

; At te time o5 te a55eted ody ti""ue in5lammation7 te di"ea"e

eome" atie. And wen te ti""ue in5lammation "u"ide"7 te

di"ea"e in an inatie "tate <in remi""ion=. +emi""ion an our

"pontaneou"ly or wile underoin treatment7 and an la"t 5or week" to

year".

; 'n ti" period o5 remi""ion7 er "ymptom" di"appeared and patient"

enerally 5eel ealtier. '5 te di"ea"e eome" atie aain <relap"e=7

te "ymptom" reappear.

5



6



2. > iano"e : yper"en"itiity 7 +eumatoid Artriti"

i55erent iano"e : eart Artriti"7 Alleri7 A"ma Bronkial7 Sok

Ana5ilaktik7 +eumatoid %eer

7



3. ?SA'7 @ortiko"teroid7 A"pirin and &A+

4. )iin andreeni ormone 7 epineprine7 anti i"tamin and anti

in5lammation non "teroid

STEP !

8



1. ; 'n5lammation +eumatoid artriti" i" on oin and "preadin to te

"truture" o5 te 6oint" and "urroundin area" inludin 6oint artilae and

5irou" 6oint ap"ule. !iament and tendon in5lammation. 'n5lammation i"arateried y te aumulation o5 wite lood ell"7 atiation o5

omplement7 eten"ie paoyto"i" and te 5ormation o5 "ar ti""ue.

Croni in5lammation will au"e ypertropy and tikened "ynoial

memrane7 au"in lood 5low re"i"tane wi au"e" ell nero"i" and

in5lammatory re"pon"e ontinue". -ikened "ynoium ten oated y

ranular ti""ue alled anu". anu" an "pread trouout te 6oint "o tat

te more "timulatin in5lammation and "arrin. -i" proe"" i" "low

damae 6oint" and au"e "eere pain and de5ormity.

; At te time o5 te a55eted ody ti""ue in5lammation7 te di"ea"e eome"

atie. And wen te ti""ue in5lammation "u"ide"7 te di"ea"e in aninatie "tate <in remi""ion=. +emi""ion an our "pontaneou"ly or wile

underoin treatment7 and an la"t 5or week" to year". 'n ti" period o5

remi""ion7 er "ymptom" di"appeared and patient" enerally 5eel ealtier.

'5 te di"ea"e eome" atie aain <relap"e=7 te "ymptom" reappear.

)ene"i" return o5 atie attak" and "ymptom" o5 reumatoid artriti"

known a" 5lare". +eumatoid artriti" di"ea"e our"e arie" on one patient

to anoter7 in wi period" o5 5lare" and remi""ion" "eemed typial 5or

ea patient. Wen te di"ea"e i" atie7 te "ymptom" an inlude 5atiue7

lo"" o5 appetite7 i 5eer7 pain and "ti55ne"" in mu"le" and 6oint".

&u"le and 6oint "ti55ne"" are u"ually mo"t prealent in te mornin or

a5ter a lon time do not do te moement. 'n5lammation o5 te 6oint"<artriti"= enerally ourred durin 5lare". At te time te"e 5lare" 6oint"

eome red7 "wollen7 pain5ul and tender. -i" our" eau"e te inner

linin o5 te a 6oint" <"ynoium= eome" in5lamed7 re"ultin in te

prodution o5 6oint 5luid <"ynoial 5luid= oerload. oket" 6oint" al"o

tikened due to in5lammation <"ynoiti"=.

; 'n reumatoid artriti"7 te 6oint" eome in5lamed wit "imitri"

pattern <ot "ide" o5 te ody under attak=. "ually a55eted are te

"mall 6oint" o5 te and" and wri"t". Simple moement" in eeryday

atiitie"7 "u a" openin te door kno or rotate te lid eome"

di55iult wen te 5lare". Small 6oint" o5 te le" are al"o Duite o5ten

epo"ed to attak. Sometime" only one 6oint i" in5lamed. Wen ti"

appen"7 it ould mat artriti" in5lammation "u a" tat au"ed y

out or 6oint in5etion. Croni in5lammation an au"e damae to

ody ti""ue7 artilae and one ard. -i" will re"ult in te lo"" o5

artilae7 ero"ion and weakenin ot one" and mu"le". Wat

appen" ten i" te ane in te 5orm o5 6oint <de5ormation=7

de"trution and lo"" o5 odily 5untion".

9



; Beau"e reumatoid artriti" i" a "y"temi di"ea"e7 te in5lammationan a55et oran" or oter ody part" out"ide te 6oint. 'n5lammation o5

te eye" and mout land" an lead to drout in part" o5 te ody and

i" known a" S6oren" "yndrome. Artriti" in5lammation o5 te linin o5

te lun" <pleuri"y= au"in e"t pain wen takin a deep reat or

ouin. !un ti""ue it"el5 may deelop in5lammation and "ometime"

rai"ed ump" in5lammation <reumatoid nodule"= in te lun".

'n5lammation o5 te outer linin o5 te eart <periarditi"= an lead to

e"t pain tat typially ane" in inten"ity wen lyin down or

endin 5orward. +eumatod di"ea"e an derea"e te numer o5 red

lood ell" <anemia=7 and wite lood ell". erea"ed wite lood ell

ount" a""oiated wit an enlared "pleen <known a" %elty" "yndrome=and an inrea"e te ri"k o5 in5etion". !ump" under te "kin

<reumatoid nodule"= an our around te elow" and 5iner" are

o5ten under pre""ure. Altou te"e ump" u"ually do not au"e

"ymptom"7 ut "ometime" an e in5eted

- -ype ' yper"en"itiity

-e"t allery <yper"en"itiity type '= on te "kin.

-ype ' yper"en"itiity i" al"o known a" diret or anapylati

yper"en"itiity. -i" reation i" a""oiated wit te "kin7 eye"7

na"oparyn7 ronkopulmonari network7 and a"trointe"tinal trat.-i" reation an re"ult in "ymptom" ranin 5rom minor di"om5ort

to deat. -e reation time rane" 5rom 1$;30 minute" a5ter epo"ure

to te antien7 ut "ometime" al"o may eperiene delay" in te "tart

up to 10;12 our". -ype ' yper"en"itiity mediated y

immunoloulin E <'E=. -e main ellular omponent in ti" reation

i" te ma"t ell" or a"opil". -i" reation ampli5ied and in5luened

y platelet"7 neutropil"7 and eo"inopil".

iano"ti te"t" tat an e u"ed to detet type ' yper"en"itiity i" a

"kin te"t <prik and intradermal= and E!'SA to mea"ure total 'E and

"pei5i 'E antiodie" to 5it te alleren <antien "pei5i alleren"=

are "u"peted. 'E leel" i" one o5 te marker" o5 allerie" due to

yper"en"itiity to te part" tat are not epo"ed diretly to te

alleren=. oweer7 it an al"o e due to an inrea"e in 'E non;atopi

di"ea"e" "u a" worm in5etion"7 myeloma7 et. &ediation tat an

e taken to addre"" te type ' yper"en"itiity i" to u"e an anti;

i"tamine to lok i"tamine reeptor"7 u"e o5 immunoloulin )

<')=7 ypo"en"itiation <immunoterapy or de"en"itiation= 5or "ome

"pei5i allery.

10



- yper"en"itiity -ype ''

empiu"7 "ample type '' yper"en"itiity in do".

-ype '' yper"en"itiity au"ed y antiodie" in te 5orm o5

immunoloulin ) <')= and immunoloulin E <'E= aain"tantien" on te ell "ur5ae and etraellular matri. -e damae

will e limited or "pei5i to ell" or ti""ue" tat are diretly related

to te"e antien". 'n eneral7 antiodie" tat diretly interat wit

ell "ur5ae antien" would e patoeni and au"e damae to te

taret ell.

yper"en"itiity reation" may inole omplement <or ro"";

reation"= tat ind" to te antiody "o tat ell" an al"o au"e

ti""ue damae. Some type" o5 yper"en"itiity type '' i":

empiu" <') reat" wit intraellular ompound" etween

epidermal ell"=7Autoimmune emolyti anemia <triered y dru" "u a"

peniillin tat an e attaed on te "ur5ae o5 red lood ell"

and at" a" a apten 5or prodution o5 antiodie" ten ind to te

"ur5ae o5 red lood ell" and au"e" ly"i" o5 red lood ell"=7 and

)oodpa"ture" "yndrome <') reat" wit te "ur5ae memrane

o5 te lomeruli7 au"in kidney damae=.

- -ype ''' yper"en"itiity

-ype ''' yper"en"itiity i" an immune omple yper"en"itiity.

-i" i" due to te depo"ition o5 antien;antiody omplee" were

"mall and di""oled in te network. 't i" arateried y te on"et o5 in5lammation or in5lammation. 'n normal ondition"7 antien;

antiody omplee" produed in lare Duantitie" and alane will e

leared y te paoyte". oweer7 "ometime"7 te pre"ene o5

ateria7 iru"e"7 enironmental7 or antien <5unal "pore"7 eetale

material7 or animal"= tat per"i"tent will make te ody

automatially produe" antiodie" to te 5orein "u"tane7 au"in

te preipitation o5 te antien;antiody omplee" ontinuou"ly. 't

al"o our" in patient" wit autoimmune di"ea"e". epo"ition o5

antien;antiody omplee" will e "pread on te memrane and

atie "eretion in te "mall annel tat an a55et multiple oran"7

11



"u a" te "kin7 kidney"7 lun"7 6oint"7 or in part" o5 te rain

oroid pleu".

-e patoene"i" o5 immune omple on"i"t" o5 two a"i pattern"7

ie immune omplee" due to ee"" antien and antiody immune

omplee" due to ee"". Croni antien ee"" will au"e pain"erum <"erum "ikne""= wi an lead to artriti" or

lomerulonepriti". Beau"e ee"" antiody immune omplee"

al"o alled Artu" reation7 au"ed y epo"ure to low do"e" o5

antien tat our" in a lon time "o tat indue te on"et o5 omple

and ee"" antiody. Some eample" o5 te ill e55et" o5 Artu"

reation i" te "pore" o5 A"perillu" laatu" and A. 5umiatu" wi

au"e pain in te lun 5ield worker" weat <malt= and "pore" o5

eniillium a"ei lun ee"e maker".

- -ype '/ yper"en"itiity Fedit G edit "oureH

&ani5iation lun iop"ie" 5rom patient" wit yper"en"itiity

pneumoniti" u"in mirorap".

-ype '/ yper"en"itiity known a" ell;mediated yper"en"itiity or

delayed type <delayed;type=. -i" reation our" eau"e te

atiity o5 ti""ue de"trution y - ell" and maropae". 't take" a

lon time in ti" reation to te atiation and di55erentiation o5 -

ell"7 te "eretion o5 ytokine" and emokine"7 a" well a" te

aumulation o5 maropae" and oter leukoyte" in te epo"ed

area. Some ommon eample" o5 yper"en"itiity type '/

yper"en"itiity pneumoniti"7 ontat yper"en"itiity <ontat

dermatiti"=7 and roni delayed type yper"en"itiity reation

<delayed type iper"en"itiity7 -=. -ype '/ yper"en"itiity an

e rouped into tree ateorie" a"ed on te "tart time o5 te on"et

o5 "ymptom"7 and linial and i"toloial appearane.

12



2. iano"e : +eumatoid artriti" wit yper"en"itiity "ide e55et

I= Ba"ed on te Coom" and )ell7 te type o5 yper"en"itiity are :

a. -ype 1 yper"en"itiity

-ype 1 yper"en"itiity reation i" a re"pon"e to ti""ue tat our" due

to ro""linkin etween alleren" and 'E. -i" reation an e re5erred to

al"o a" a Duik reation7 alleri reation7 or anapylati reation". A

ommon meani"m o5 ti" reation a" 5ollow":

; Alleren" ro""linked wit 'E

; &a"t ell" and a"opil" "erete a"oatie amine" and oter

emial mediator"

; Ari"e mani5e"tation&ani5e"tation" ari"in 5rom ti" reation in te 5orm o5 anapylai"7

urtiaria7 ronial a"tma or atopi dermatiti".

. yper"en"itiity -ype 3

-ype 3 yper"en"itiity reation" our due to depo"ition o5 immune

omplee" <antien;antiody= are di55iult di5ao"it "o will atiate

omplement and polymorponulear leukoyte" aumulate in ti""ue".

-i" reation an al"o e alled an immune omple;mediated reation".

-i" reation on"i"t" o5 two 5orm" o5 reation7 namely: Sy"temi 'mmune

13



omple reation" <"erum "ikne""= and !oal 'mmune Sy"tem reation

<Artu"=. -e meani"m o5 ti" reation i" enerally a" 5ollow":

; -e 5ormation o5 antien;antiody omplee" are di55iult di5ao"it

; Enalin te omplement

; Attratin ?eutropil" ; -e relea"e o5 ly"o"omal enyme"

; Ependiture emial mediator"

; Ari"e mani5e"tation

&ani5e"tation" au"ed y ti" reation an e Artu" reation7 "erum

"ikne""7 !ES7 A+7 lomerulonepriti"7 and pneumoniti".

3. o""iility ny.Sara on"ume anale"i" ae "ide e55et" yper"en"itiity.

ny.Sara aoided 5or not takin a"pirin and oliation to on"ume

immuno"uppre""ant7 ?SA'"7 and &A+. 'n te a"e o5 a"pirin in )out

Artriti" +eumatoid Artriti" ontraindiated wile on a"pirin i" anindiation.

4. '5 an anapylati reation to te ien "u;adreneri and ien

a""i"tane in reatin and make "ure te patient" le" ier tan your

ead. 'n a"e o5 "ortne"" o5 reat an e ien epineprine7

ortio"teroid"7 anti;in5lammatory7 and i5 it ad known te patient ad a

preiou" dru allerie"7 out er "tandard" are away 5rom alleren" and

ien antii"tamine"

STEP 5 'Learnin( O)*ec"i+e,

1. Eplain in detail" aout reumatoid artriti"<+A= and "y"temi lupu"erytemato"u" <S!E=J

2. Eplain aout onept o5 autoimunJ3. Wat i" te mani5e"tation o5 Steen ,on"on" Syndrome7 eno Solein

urpura7 and ,uenil roni artiti"4. Wat i" te ompliation o5 S!E7 "lenoderma7 poliartriti" nodu"a7 a"uliti"

lupu"

14



STEP -

. ermono Baman7 Sutarno7 ra"ena.')7 dkk. Buku Ajar Hemato

Onkolo! Anak "K UI#'katan okter 'ndone"ia 2012

Sudoyo Aru.W7 Setiyoadi Baman7 Alwi 'dru"7dkk. Ilmu $en%ak!t &alam '!l!d

Ed!! *#

15



STEP .

1.

R#e%/a"oi$ A"ri"i

eople ae lon 5eared reumatoid artriti" <ommonly alled +A= a" oneo5 te mo"t di"alin type" o5 artriti". -e ood new" i" tat te outlook a" reatly improed 5or many people wit newly diano"ed <deteted= +A.

16



K5 our"e7 +A remain" a "eriou" di"ea"e7 and one tat an ary widely in"ymptom" <wat you 5eel= and outome". Een "o7 treatment adane"ae made it po""ile to "top or at lea"t "low te prore""ion <wor"enin=o5 6oint damae. +eumatoloi"t" now ae many new treatment" tat

taret te in5lammation tat +A au"e". -ey al"o under"tand etter wenand ow to u"e treatment" to et te e"t e55et".

Fa" ac"

+A i" an autoimmune di"ea"e. A 5aulty immune "y"tem <te ody" de5en"e

"y"tem= trier" it.

+A i" te mo"t ommon type o5autoimmune artriti". At lea"t 1.3million .S. adult" ae +A.-reatment" ae improed reatly and elp many o5 to"e a55eted.

+eumatoloi"t" are dotor" wo ae te eperti"e to orretly diano"e

ti" di"ea"e and to o55er patient" te mo"t adaned treatment".

#a" i r#e%/a"oi$ ar"#ri"i

+A i" a roni <lon;term= di"ea"e tat au"e" pain7 "ti55ne""7 "wellin

and limited motion and 5untion o5 many 6oint". Wile +A an a55et any

6oint7 te "mall 6oint" in te and" and 5eet tend to e inoled mo"t o5ten.

'n5lammation "ometime" an a55et oran" a" well7 5or in"tane7 te eye"

or lun".

-e "ti55ne"" "een in atie +A i" mo"t o5ten wor"t in te mornin. 't may

la"t one to two our" <or een te wole day=. Sti55ne"" 5or a lon time inte mornin i" a lue tat you may ae +A7 "ine 5ew oter artriti

di"ea"e" eae ti" way. %or in"tane7 o"teoartriti" mo"t o5ten doe" not

au"e proloned mornin "ti55ne"".

17

http://www.rheumatology.org/practice/clinical/patients/diseases_and_conditions/osteoarthritis.asp





Kter "in" and "ymptom" tat

an our in +A inlude: !o"" o5

enery

!ow5eer

"!o""o5appetitery eye" and mout 5rom a related ealt prolem7 S6oren" "yndrome

%irm lump"7 alled reumatoid nodule"7 wi row eneat te "kin in plae" "u a" te elow and and"

#a"ca%er#e%/a"oi$

ar"#ri"i+A i" an

autoimmune

di"ea"e. -i"

mean" tat

ertain ell" o5

te immune

"y"tem do not

work properly

and "tart

attakin ealty

ti""ue" L te

6oint" in +A.

-e au"e o5 +A

i" not known.

*et7 new

re"ear i"

iin u" a

etter idea o5

wat make" teimmune "y"tem

attak te ody

and reate

in5lammation. 'n

+A7 te 5ou" o5

te in5lammation

i" in te

"ynoium7 te

ti""ue tat line"

te 6oint.

'mmune ell"

relea"e in5lammation;au"in emial".

-e"e emial" an damae artilae <te

ti""ue tat u"ion" etween 6oint"= and

one.

18

http://www.rheumatology.org/practice/clinical/patients/diseases_and_conditions/sjogrens.asp

http://www.rheumatology.org/practice/clinical/patients/diseases_and_conditions/sjogrens.asp



-e normal 6oint "truture appear" on tele5t. Kn te rit i" te 6oint wit reumatoidartriti". +A au"e" "ynoiti"7 pain and"wellin o5 te "ynoium <te ti""ue tatline" te 6oint=. -i" an make artilae <teti""ue tat u"ion" etween 6oint"= and oneerode7 or wear away.

Kter tin" likely play a role in +A a" well. %or in"tane7 ene" tata55et te immune "y"tem may make "ome people more prone toettin +A.

#o (e" r#e%/a"oi$ ar"#ri"i

+A i" te mo"t ommon 5orm o5 autoimmune artriti"7 a55etin more

tan 1.3 million Amerian". K5 te"e7 aout ($M are women. 'n 5at7 1>

3M o5 women may et reumatoid artriti" in teir li5etime. -e di"ea"e

mo"t o5ten ein" etween te 5ourt and "it deade" o5 li5e. oweer7

+A an "tart at any ae.

19



o4 i r#e%/a"oi$ ar"#ri"i $ia(noe$

+A an e ard to detet eau"e it may ein wit "utle "ymptom"7 "u a" ay

6oint" or a little "ti55ne"" in te mornin. Al"o7 many di"ea"e" eae like +A early on.

%or ti" rea"on7 i5 you or your primary are py"iian tink" you ae +A7 you "ould

"ee a reumatoloi"t. A reumatoloi"t i" a py"iian wit te "kill and knowlede to

rea a orret diano"i" o5 +A and to make te mo"t "uitale treatment plan.

iano"i" o5 +A depend" on te "ymptom" and re"ult" o5 a py"ial eam7 "u a"warmt7 "wellin and pain in te 6oint". Some lood te"t" al"o an elp on5irm +A.-elltale "in" inlude:

Anemia <a low red lood ell ount=

+eumatoid 5ator <an antiody7 or lood protein7 5ound in aout 80M o5 patient"wit +A in time7 ut in a" 5ew a" 30M at te "tart o5 artriti"=

Antiodie" to yli itrullinated peptide" <piee" o5 protein"=7 or anti;CC 5or "ort

<5ound in #0> (0M o5 patient" wit +A=Eleated erytroyte "edimentation rate <a lood te"t tat7 in mo"t patient" wit +A7on5irm" te amount o5 in5lammation in te 6oint"=

N;ray" an elp in detetin +A7 ut may not "ow anytin anormal in early

artriti". Een "o7 te"e 5ir"t N;ray" may e u"e5ul later to "ow i5 te di"ea"e i"

prore""in. K5ten7 &+' and ultra"ound "annin are done to elp 6ude te "eerity o5

+A.

-ere i" no "inle te"t tat on5irm" an +A diano"i" 5or mo"t patient" wit ti"

di"ea"e. <-i" i" aoe all true 5or patient" wo ae ad "ymptom" 5ewer tan "i

mont".= +ater7 a dotor make" te diano"i" y lookin at te "ymptom" and re"ult"

5rom te py"ial eam7 la te"t" and N;ray".

o4 i r#e%/a"oi$ ar"#ri"i "rea"e$

-erapy 5or +A a" improed reatly in te pa"t 30 year". Current treatment" ie mo"t

patient" ood or eellent relie5 o5 "ymptom" and let tem keep 5untionin at7 or near7

normal leel". Wit te rit mediation"7 many patient" an aiee Oremi""ionP L

tat i"7 ae no "in" o5 atie di"ea"e.

-ere i" no ure 5or +A. -e oal o5 treatment i" to le""en your "ymptom" and poor5untion. otor" do ti" y "tartin proper medial terapy a" "oon a" po""ile7 e5ore

your 6oint" ae la"tin damae. ?o "inle treatment work" 5or all patient". &any

people wit +A mu"t ane teir treatment at lea"t one durin teir li5etime.

)ood ontrol o5 +A reDuire" early diano"i" and7 at time"7 are""ie treatment. -u"7

patient" wit a diano"i" o5 +A "ould ein teir treatment wit di"ea"e;modi5yin

antireumati dru" L re5erred to a" &A+". -e"e dru" not only reliee

"ymptom" ut al"o "low prore""ion o5 te di"ea"e. K5ten7 dotor" pre"rie &A+"

alon wit non"teroidal anti;in5lammatory dru" or ?SA'" andQor low;do"e

ortio"teroid"7 to lower "wellin7 pain and 5eer. &A+" ae reatly improed te"ymptom"7



5untion and Duality o5 li5e 5or nearly all patient" wit +A. A"k your reumatoloi"taout te need 5or &A+ terapy and te ri"k" and ene5it" o5 te"e dru".

Common &A+" inlude metotreate <rand name" inlude +eumatreR and

%oleR=7 le5lunomide <Araa=7 ydroyloroDuine <laDuenil= and "ul5a"alaine

<Aul5idine=. Klder &A+" inlude old7 ien a" a pill L aurano5in <+idaura= L

or more o5ten a" an in6etion into a mu"le <"u a" &yory"ine=. -e antiioti

minoyline <e..7 &inoin7 ynain and /etrin= al"o i" a &A+7 a" are te

immune "uppre""ant" aatioprine <'muran= and ylo"porine <Sandimmune and

?eoral=. -e"e tree dru" and old are rarely pre"ried 5or +A te"e day" eau"e

oter dru" work etter or ae 5ewer "ide e55et".

atient" wit more "eriou" di"ea"e may need mediation" alled ioloi re"pon"e

modi5ier" or Oioloi aent".P -ey an taret te part" o5 te immune "y"tem and te

"inal" tat lead to in5lammation and 6oint and ti""ue damae. -e"e mediation" areal"o &A+". %A;approed dru" o5 ti" type inlude aataept <Krenia=7

adalimuma <umira=7 anakinra <@ineret=7 ertoliuma <Cimia=7 etanerept <Enrel=7

olimuma <Simponi= in5liima <+emiade=7 rituima <+ituan= and toiliuma

<Atemra=. &o"t o5ten7 patient" take te"e dru" wit metotreate7 a" te mi o5

mediine" i" more elp5ul.

-e e"t treatment o5 +A need" more tan mediine" alone. atient eduation7 "u

a" ow to ope wit +A7 al"o i" important. roper are reDuire" te eperti"e o5 a

team o5 proider"7 inludin reumatoloi"t"7 primary are py"iian"7 and py"ial

and oupational terapi"t". *ou will need 5reDuent i"it" trou te year wit

your reumatoloi"t. -e"e ekup" let your dotor trak te our"e o5 your

di"ea"e and ek 5or any "ide e55et" o5 your mediation". *ou likely al"o will

need to repeat lood te"t" and N;ray" or ultra"ound" 5rom time to time.

#a" i "#e )roa$er #ea&"# i/ac" o r#e%/a"oi$ ar"#ri"i

+e"ear "ow" tat people wit +A7 mainly to"e wo"e di"ea"e i" not wellontrolled7 ae a ier ri"k 5or eart di"ea"e and "troke. -alk wit your dotoraout te"e ri"k" and way" to lower tem.

Li+in( 4i"# r#e%/a"oi$ ar"#ri"i

't i" important to e py"ially atie mo"t o5 te time7 ut to "ometime" "ale ak

atiitie" wen te di"ea"e 5lare". 'n eneral7 re"t i" elp5ul wen a 6oint i" in5lamed7

or wen you 5eel tired. At te"e time"7 do entle rane;o5;motion eeri"e"7 "u a"

"tretin. -i" will keep te 6oint 5leile.

Wen you 5eel etter7 do low;impat aeroi eeri"e"7 "u a" walkin7 and

eeri"e" to oo"t mu"le "trent. -i" will improe your oerall ealt and redue

pre""ure on your 6oint". A py"ial or oupational terapi"t an elp you 5ind wi

type" o5 atiitie" are e"t 5or you7 and at wat leel or pae you "ould do tem.

http://www.rheumatology.org/practice/clinical/patients/medications/methotrexate.asp

http://www.rheumatology.org/practice/clinical/patients/medications/leflunomide.asp

http://www.rheumatology.org/practice/clinical/patients/medications/hydroxychloroquine.asp

http://www.rheumatology.org/practice/clinical/patients/medications/sulfasalazine.asp

http://www.rheumatology.org/practice/clinical/patients/medications/gold.asp

http://www.rheumatology.org/practice/clinical/patients/medications/minocycline.asp

http://www.rheumatology.org/practice/clinical/patients/medications/azathioprine.asp

http://www.rheumatology.org/practice/clinical/patients/medications/cyclosporine.asp

http://www.rheumatology.org/practice/clinical/patients/medications/abatacept.asp


http://www.rheumatology.org/practice/clinical/patients/medications/tocilizumab.asp


http://www.rheumatology.org/practice/clinical/patients/medications/methotrexate.asp

http://www.rheumatology.org/practice/clinical/patients/medications/leflunomide.asp

http://www.rheumatology.org/practice/clinical/patients/medications/hydroxychloroquine.asp

http://www.rheumatology.org/practice/clinical/patients/medications/sulfasalazine.asp

http://www.rheumatology.org/practice/clinical/patients/medications/gold.asp



http://www.rheumatology.org/practice/clinical/patients/medications/azathioprine.asp

http://www.rheumatology.org/practice/clinical/patients/medications/cyclosporine.asp






%indin tat you ae a roni illne"" i" a li5e;anin eent. 't an au"e worry

and "ometime" 5eelin" o5 i"olation or depre""ion. -ank" to reatly improed

treatment"7 te"e 5eelin" tend to derea"e wit time a" enery improe"7 and pain

and "ti55ne"" derea"e. i"u"" te"e normal 5eelin" wit your ealt are proider".

-ey an proide elp5ul in5ormation and re"oure".

Poin" "o re/e/)er

Ne4er "rea"/en" are eec"i+e6 +A dru" ae reatly improed outome" 5or patient". %or mo"t people wit +A7 early treatment an ontrol 6oint pain and"wellin7 and le""en 6oint damae.

See7 an e8er" in ar"#ri"i9 a r#e%/a"o&o(i"6 Eperti"e i" ital to make an earlydiano"i" o5 +A and to rule out di"ea"e" tat mimi +A7 tu" aoidin unneeded te"t"and treatment". A dotor wo i" an epert in +A al"o an de"in a u"tomiedtreatment plan tat i" e"t "uited 5or you.

-ere5ore7 te reumatoloi"t7 workin wit te primary are py"iian and oterealt are proider"7 "ould "uperi"e te treatment o5 te patient wit +A.

S"ar" "rea"/en" ear&:6 Studie" "ow tat people wo reeie early treatment 5or +A5eel etter "ooner and more o5ten7 and are more likely to lead an atie li5e. -ey al"oare le"" likely to ae te type o5 6oint damae tat lead" to 6oint replaement.



S:"e/ic Eri"e/a"o% L%%

261 Deini"ion o LES6

Sy"temi !upu" Erytemato"u" <!ES= i" an autoimmune reumati di"ea"e

arateried y wide"pread in5lammation7 wi a55et any oran or "y"tem in

te ody. -e di"ea"e i" a""oiated wit autoantiodie" and immune omple

depo"ition re"ultin in damae 6arinan1.

262 Ei$e/io&o(: LES6

-i" di"ea"e attak" te youn woman wit a peak inidene 1$;40 year" o5

ae durin te reprodutie period wit 5emale and male ratio $: 1. 'n te la"t

30 year"7 !ES a" eome one o5 te ma6or di"ea"e" o5 reumati di"ea"e" in

te world. !ES prealene in di55erent ountrie" arie" etween 2.9 Q 100000;

400 Q 1007000. !ES i" more ommon in ertain rae" like te nero7 Cina7 and

may e ilippine"2.

263 E"ioa"#o(enei LES6

Etiopatoene"i" o5 !ES i" "till not known learly7 were tere i" plenty o5

eidene tat te patoene"i" o5 te !ES i" multi5aktoral "u a" eneti

5ator"7 enironmental 5ator"7 and ormonal 5ator" on te re"pon"e

imun.%aktor eneti" play" a role in many lupu" patient" wit an inrea"ed ri"k

in "ilin" and monoyoti twin". +eent re"ear "ow" tat many ene" tat

play a role7 e"peially te ene tat ode" 5or te element" o5 te immune

"y"tem. Alleedly a""oiated wit ene;"pei5i immune re"pon"e" in omple

la"" '' ma6or i"tokompailita"7 namely !A;+2 and !A;+3 and te

omplement omponent" tat play a role in te initial pa"e o5 te reation

ind omplement <ie C1D7 C1r7 C1"7 C47 and C2= a" een proen. Kter ene"

tat ein to ome into play i" te ene enodin te - ell reeptor7

immunoloulin" and ytokine".

Kter "tudie" on eneti 5ator" tat "tudie" related to !A <uman !eukoyte

Antien"= wi "upport" te onept tat te &C ene" <&a6or

i"toompatiility Comple= reulate" te prodution o5 "pei5i

autoantiodie". eople wit lupu" <approimately #M= inerited de5iieny o5

omplement omponent"7 "u a" C27 C47 or C1D14;1$. Complement

de5iieny an impair irulation relea"e o5 immune omplee" y

mononulear 5ao"ito"it "y"tem tat elp" te ti""ue depo"ition. C1D de5iieny

au"e" paoyti ell" 5ail to lean up nulear apoptoti ell" "o tat te

omponent will au"e an immune re"pon"e.



Enironmental 5ator" an e triered in patient" wit lupu"7 "u a"

ultraiolet radiation7 toao7 dru"7 iru"e". / lit lead" to a lo"" o5 "el5;

immunity and tolerane eau"e it au"e" apopto"i" o5 keratinoyte". 'n

addition7 / lit au"e" te relea"e o5 immune mediator" in patient" wit

lupu"7 and play" a role in te indution pa"e yann diretly alter ell

?A7 a" well a" a55etin normal ell" wen imunoreulator wi elp

"uppre"" te ourrene o5 anormalitie" in in5lammatory kulit14;1#. Kter

enironmental 5ator" are "mokin ait" wi "ow" tat "moker" ae a

ier ri"k o5 lupu"7 a""oiated wit te "u"tane" ontained in toao tat

lipoeni amino aromatik13. Al"o ie" an oeriew o5 te in5luene o5 dru"

arie" in patient" wit lupu". -e in5luene o5 dru" one o5 wi an inrea"e

keratinoyte apopto"i". Kter enironmental 5ator"7 namely te role o5

in5etiou" aent"7 e"peially iru"e" an e 5ound in people wit lupu". -eruella iru"7 ytomealoiru"7 an a55et te epre""ion o5 ell "ur5ae and

apopto"i"1$;1#. -e tird 5ator tat a55et" te patoene"i" o5 lupu" tat

ormonal 5ator". -e ma6ority o5 ti" di"ea"e a55et" youn women and "ome

re"ear "ue"t" tere i" a reiproal relation"ip etween te leel" o5

ormone"troen te immune "y"tem. E"troen polylonal B ell atiation7

re"ultin in ee""ie prodution o5 autoantiodie" in lupu" patient" !ES18;

20.Autoantiodi ten 5ormed to eome nulear antien" <A?A and anti;

?A=. 'n addition7 antiodie" aain"t oter ell "truture" "u a" erytroyte"7

platelet" and po"polipid". Autoantiodie" are inoled in te 5ormation o5

immune omplee"7 omplement atiation 5ollowed y a55etin te

in5lammatory re"pon"e in many ti""ue"7 inludin te "kin and in6al21;

26! C&inica& Manie"a"ion LES6

-e linial mani5e"tation" o5 ti" di"ea"e ary dependin on te oran

inoled wi an inole many oran" in te uman ody wit a omple

linial our"e7 ily ariale7 an e arateried y aute attak"7 te

period o5 atie7 omple7 or remi""ion and o5ten te initial "tate i" not

reonied a" an !ES. -i" an appen eau"e te linial mani5e"tation" o5

te di"ea"e i" o5ten not te a"e !ES "imultaneou"ly. A per"on an e 5or

"eeral year" omplained o5 6oint pain tat moed witout any oter

omplaint". -i" wa" 5ollowed y oter linial mani5e"tation" "u a"

poto"en"itiity7 and "o on tat will eentually meet te riteria !ES.

26!61 Manie"a"ion ;on"i"%iona&16

%atiue i" a ommon omplaint in patient" wit !ES and u"ually preede"

oter linial mani5e"tation" .. %atiue i" "omewat di55iult to a""e"" eau"e

many oter ondition" tat an au"e 5atiue "u a" anemia7 inrea"ed

workload7 p"yoloial on5lit"7 a" well a" te u"e o5 dru" "u a"



predni"one. '5 te 5atiue au"ed y te di"ea"e atiity !ES7 oter

ine"tiation" are reDuired leel" o5 low "erum C3. -e "train o5 te di"ea"e

re"pond to "teroid" or eeri"e.

Weit lo"" 5ound in mo"t patient" wit !ES and our witin a 5ew mont" e5ore te diano"i" i" made. Weit lo"" an e au"ed y derea"ed appetite

or a"trointe"tinal "ymptom" au"ed.

%eer a" one o5 te !ES on"titutional "ymptom" are di55iult to di"tinui"

5rom oter au"e" "u a" in5etion due to a temperature reater tan 40 C in

te a"ene o5 eidene o5 oter in5etion" "u a" leukoyto"i". !ES 5eer i"

u"ually not aompanied y "ierin.

26!62 Manie"a"ion M%7%&o7e&e"a&126

'n patient" wit !ES7 mu"ulo"keletal mani5e"tation" 5ound polyartriti"7

u"ually "ymmetrial wit epi"ode" o5 artralia in 90M o5 a"e". 'n $0M o5

a"e" an e 5ound mornin "ti55ne""7 tendoniti" o5ten our" wit no ero"ion

due to 6oint "uluation 6oint". Kter "ymptom" tat an e 5ound in te 5orm

o5 o"teonero"i" were 5ound in $;10M o5 a"e" and i" u"ually a""oiated wit

"teroid terapy.

'n addition7 tere i" al"o myalia ourrin in #0M o5 a"e"7 ut myo"iti"

our" in patient" wit !ES T$M o5 a"e". &yopaty an al"o e 5ound7

u"ually a""oiated wit "teroid terapy and CloroDuine. K"teoporo"i" i"ommon and i" a""oiated wit di"ea"e atiity and "teroid u"e.

26!63 Manie"a"ion ;%&i"126

Skin di"order" are o5ten 5ound in te !ES i" poto"en"itiity7 utter5ly ra"7

malar ra"e"7 roni di"oid le"ion"7 alopeia7 panikuliti"7 p"oria5orm le"ion"

and oter". 'n addition7 te "kin an al"o e 5ound "in" o5 utaneou" a"uliti"7

"u a" +aynaud" penomenon7 liedo retiulari"7 5iner uler"7 anrene.

26!6! Manie"a"ion ;ar$io+a7%&ar

Cardioa"ular anormalitie" in !ES inlude periardial di"ea"e7 an e mild

periarditi"7 periardial e55u"ion7 periardial tikenin up. &yoarditi" an e

5ound in 1$M o5 a"e"7 i" arateried y tayardia7 arrytmia7 + interal

etend"7 ardiomealy to eart 5ailure.

!iman;Sa" endoarditi"7 o5ten undiano"ed in te lini7 ut te data

autop"y et $0M !ES wit !iman;Sa" endoarditi". -e pre"ene o5

eetation ale wit 5eer "ould e "u"peted te po""iility o5 aterial

endoarditi". Women wit !ES ae oronary eart di"ea"e ri"k $;#M ier

tan normal women. 'n women aed 3$;44 year"7 ti" ri"k inrea"e" to $0M.



26!65 Manie"a"ion o L%n(

!un di"order" in te !ES are o5ten "ulinial "o radiorapi and "pirometry

"ould e per5ormed in patient" !ES wit ou7 "ortne"" o5 reat or oter

re"piratory di"order". leuri"y and pleuriti pain an e 5ound in #0M o5 a"e".leural e55u"ion an e 5ound in 30M o5 a"e"7 ut it i" u"ually mild and not

linially "ini5iant. 'nter"titial 5iro"i"7 pulmonary a"uliti" and pneumoniti"

an e 5ound in 20M o5 a"e"7 ut linially it i" o5ten di55iult to di"tinui"

5rom pneumonia and one"tie eart 5ailure. ulmonary yperten"ion i" o5ten

5ound in patient" wit antipo"polipid "yndrome. atient" wit pleuriti pain

and pulmonary yperten"ion "ould e ealuated 5or te po""iility o5

antipo"polipid "yndrome and pulmonary emoli"m.

26!6- Manie"a"ion Gin*a&

enilainan renal inolement in patient" wit !ES "ould e done y a""e""in

te pre"ene Q a"ene o5 yperten"ion7 proteinuria and urinaly"i" to "ee

"ilinderuria7 urea and reatinine7 proteinuria Duantitatie7 and reatinine

learane. i"toloially7 WK "plit on $ la""e" o5 lupu" nepriti". S!E

patient" wit miro"opi ematuria and Q or proteinuria wit derea"ed )%+

"ould e on"idered 5or renal iop"y.

26!6. Manie"a"ion e/ooe"i7

'n !ES7 inrea"ed erytroyte "edimentation rate <ES+=7 wi i" aompanied y normokrom normoyti anemia au"ed y anemia o5 roni di"ea"e7

roni kidney di"ea"e7 ero"ie a"triti" wit emorrae and autoimmune

emolyti anemia.

'n addition7 leukopenia and lympopenia were al"o 5ound in $0;80M o5 a"e".

-e pre"ene o5 leukoyto"i" "ould e "u"peted te po""iility o5 in5etion.

-romoytopenia in !ES wa" 5ound in 20M o5 a"e". atient" wo initially

"owed a piture o5 idiopati tromoytopenia <'-=7 o5ten later deeloped

into te !ES a5ter anoter 5ound !ES piture.

26!6< S"r%c"%re Sara /anie"a"ion6

!ES ?europ"yiatri inolement arie" reatly7 an e a miraine7

periperal neuropaty7 to "eiure" and p"yo"i". -romoemoli di"order"

wit anti;po"polipid antiodie" may e te mo"t ommon au"e o5

ereroa"ular di"order in te !ES. eriperal neuropaty7 e"peially "en"ory

type 5ound in 10M o5 a"e".



Corelation o5 rain nere"7 rarely ditemukan.@elainan p"yiatri o5ten 5ound7

ranin 5rom aniety7 depre""ion to p"yo"i". "yiatri di"order" an al"o e

triered y "teroid terapy. Analy"i" o5 erero"pinal 5luid o5ten do not ie a

"pei5i de"ription7 eept to rule out te po""iility o5 in5etion.

Eletroenepalorapy <EE)= al"o doe" not ie a piture "pei5i. C- "an

o5 te rain i" "ometime" nee""ary to di"tinui" teir in5artion or

emorrae.

26!6= Manie"a"ion Ga"roin"e"ina&

Can e epatomealy7 adominal pain are not "pei5i7 "plenomealy7 a"epti peritoniti"7 a"uliti" me"enterial7 panreatiti". 'n addition7 tere i" al"o an

inrea"e in AS- and A!- "ould e ealuated aain"t te po""iility o5

autoimmune epatiti".

265 Dieae Ac"i+i": LES6

-o a""e"" te di"ea"e atiity o5 te !ES an e a""e""ed wit a "ore amon

oter" y u"in te S!E i"ea"e Atiity 'nde <S!EA'=7

Briti" '"le" !upu" A""e""ment )roup <B'!A)=7 Sy"temi !upu" Atiity

&ea"urement <S!A&=7 te European Con"en"u" !upu" Atiity &ea"urement<EC!A&= or !upu" Atiity 'nde <!A&= .S!E i"ea"e Atiity 'nde

<S!EA'=7 te Briti" '"le" !upu" A""e""ment )roup <B'!A)= 7 and Sy"temi

!upu" Atiity &ea"urement <S!A&= proed alid and "en"itie to !ES2#

di"ea"e atiity.

+e"ear onduted y EutUlia %reire Andrade &edeiro"7 &edeiro" !aU" Souto7

and +oana &e"Duita Cionelli in Brail in 2011 "owed tat S!E i"ea"e

Atiity 'nde <S!EA'= a" a ery i alidity ompared to te Briti" '"le"

!upu" A""e""ment )roup <B'!A)= and Sy"temi !upu" Atiity &ea"urement

<S!A&= 2#. 'n addition7 te S!E i"ea"e Atiity 'nde <S!EA'= doe" not

reDuire epen"ie and ea"y to u"e. -ere5ore7 S!E i"ea"e Atiity 'nde

<S!EA'= i" o5ten u"ed to a""e"" di"ea"e atiity in re"ear !ES2# !ES.

26- In+e"i(a"ion6

26-61 E8a/ina"ion Ro%"ine )&oo$ an$ %rine e8a/ina"ion6

'ne"tiation" were arried out on te di"ea"e Sy"temi !upu" Erytemato"u"

<!ES= i" a routine lood te"t" and urine. -e re"ult" o5 lood te"t" in patient"



wit !ES "owed emolyti anemia7 tromoytopenia7 lympopenia7 or

leukopeniaV erytroyte"edimentation rate

<ES+= inrea"e" durin atie di"ea"e7 Coom" te"t may e po"itie7 ') leel"

may e i7 te alumin;loulin ratio i" reer"ed7 and inrea"ed "erumloulin. 'n addition7 te re"ult" o5 urine in patient" wit !ES "owed

proteinuria7 ematuria7 inrea"ed reatinine7 and te di"oery Ca"t7 eme

ranular or red lood ell" in te urine.

26-62 E8a/ina"ion A%"oan"i)o$i

atoeni proe""e" ea di"ea"e i" a""oiated wit te ariou" immunoloi

proe""e"7 eiter "pei5i or non;"pei5i. -e relation"ip i" ertainly more

oiou" in autoimmune di"ea"e"7 inludin in it !ES7 +eumatoid Artriti"7

S6oren" "yndrome and "o on. -e pre"ene o5 antiodie" inludinautoantiodie" are o5ten u"ed in an e55ort to elp te diano"i" and ealuation

o5 di"ea"e prore""ion and terapy ien.

-e 5ormation o5 autoantiodie" i" Duite omple and no "inle "tudy an

eplain te wole meani"m pato5i"ioloiknya. Similarly7 te prolem

autoimmunity. Kn te latter i""ue7 "aid tere i" ao" in te entral "y"tem o5

immune tolerane in -;elper and ae irt to many ypote"e"7 amon oter

modi5iation" autoantien7 or moleular mimiry antieni "imilarity to te -;

ell epitope7 ro"";reatie peptide aain"t B;ell epitope"7 ypa"" meani"m

idiotipik7 polylonal atiation and "eainya. Kter meani"m" an al"o e

"een 5rom te point o5 ell reulatory meani"m" inter5erene eiter 5rom te

tymu" to periper. -i" me"" te etter ane ourrene in line wit te

inrea"in ae o5 te per"on.

)enerally7 autoantiodie" it"el5 doe" not immediately lead to illne"". -ere5ore7

it i" etter "een a" a marker autoantiodie" <marker"= patoloi proe"" rater

tan a" a patoloial aent. !eel" wi an o up or down an e a""oiated

wit di"ea"e atiity or a" a re"ult o5 terapeuti interention. Autoantiodila

autoantien omplee" and will ein a "erie" o5 autotoimun di"ea"e. ntil

now7 ti" ypote"i" adopted i" a new autoantiody i" "aid to ae a role in te

deelopment o5 an autoimmune reumati di"ea"e wen e wa" in"trumental in

te proe"" patoloiknya.

26-6261 an"in%c&ear an"i)o$ie

Antinulear antiody <A?A= i" a roup o5 autoantiodie" "pei5i 5or nulei

aid and nuleoprotein7 5ound in onnetie ti""ue di"ea"e "u a" S!E7

"y"temi "lero"i"7 &ied Connetie -i""ue i"ea"e <&C-= and S6ren"

"yndrome primary. A?A wa" 5ir"t di"oered y arreae" in 1948 in te

one marrow o5 patient" !ES. Wit te deelopment o5 te eamination an e



5ound immunodi55u"ion new A?A "pei5iity "u a" Sm7 nulear

rioleoprotein <n+?=7 +o Q SS;A and !a Q SS;B.

A?A an e eamined u"in immuno5luore"ene metod. A?A are u"ed a"

"reenin te"t" in onnetie ti""ue di"ea"e. Wit a ood eamination7 99M o5 patient" wit !ES "ow" a po"itie eamination7 #8M in patient" wit S6oren"

"yndrome and 40M in patient" "kleroderma.A?A al"o at 10M o5 normal

population aedX (0 year".

26-6262 An"i)o$ie "o DNA

Antiodie" to ?A <anti d";?A= an e la""i5ied into reatie antiodie"

aain"t natie ?A <doule;"tranded;?A=. Anti;d";?A po"itie patient"

wit i leel" 5ound in (3M o5 S!E and ae diano"ti and prono"ti

"ini5iane. !eel" o5 anti;d";?A wa" 5ound on te lower S6oren""yndrome7 reumatoid artriti". 'nrea"ed leel" o5 anti;d";?A "owed an

inrea"e in di"ea"e atiity. 'n !ES7 anti;d";?A a" a "tron orrelation wit

lupu" nepriti" and S!E di"ea"e atiity. Anti d";?A eamination arried out

y te metod o5 radioimmunoa""ay7 E!'SA and C.luiliae immuno5luore"en".

26-63 E8a/ina"ion ;o/&e/en

Complement i" a moleule o5 te immune "y"tem tat i" not "pei5i.

Complement" are in irulation in an inatie "tate. 'n te eent o5 atiation

y antien"7 immune omplee"7 and oter"7 will produe a ariety o5 mediator" are atie to de"troy te antien. Complement i" one enyme "y"tem

on"i"tin o5 20 pla"ma protein" and work in "eDuene <"el5;ampli5yin= a" a

model o5 lood oaulation and 5irinoly"i" a"ade.

'n !ES7 te leel" o5 C17 C47 C2 and C3 are u"ually low7 ut te normal

utaneou" lupu". @ompemen derea"ed leel" a""oiated wit te "eerity o5

S!E primarily renal ompliation". Serial o"eration" in patient" wit

eaeration7 derea"ed omplement leel" "een earlier tan linial

"ymptom".

26. Dia(noi LES

iano"i" !ES7 an e e"tali"ed a"ed on te linial piture and laoratory.

Amerian Collee o5 +eumatoloy <AC+=7 in 199(7 5iled 11 riteria 5or te

la""i5iation o5 te !ES7 wi i5 otained 5our riteria7 te diano"i" an e

e"tali"ed !ES.

-e"e riteria are:

1. malar ra".



2. di"oid ra".

3. oto"en"itiity.

4. uler" in te mout.

$. -e non;ero"ie artriti".

#. leuri"y or periarditi".

(. 'mpaired renal7 namely per"i"tent proteinuriaX 07$r Q day7 or ylindrial

ell" an e erytroyte"7 emoloin7 ranular7 tuular or ompo"ite.

8. i"order" neuroloy7 ie "eiure" or p"yo"i".

9. ematoloi i"order"7 ie emolyti anemia wit retiulo"e"7 or leukopenia

or lympopenia or tromoytopenia.

10. 'mpaired immunoloi7 ie po"itie anti;?A7 anti;Sm or po"itie or

"eroloi te"t" 5or "ypili" were 5al"e po"itie".

11. Antinulear antiody <Antinulear antiodie"7 A?A= po"itie

26< Mana(e/en" o LES in (enera&

Coun"elin and p"yo"oial interention i" ery important to e on"idered in

te manaement o5 patient" wit !ES7 e"peially in patient" wit newlydiano"ed. -i" an e aieed y diret eten"ion to te patient or to 5orm a

roup o5 people wo meet reularly to talk aout i" illne"". 'n eneral7

patient" wit !ES eperiene poto"en"itiity7 "o patient" "ould e reminded

not to e too mu epo"ed to te "un. -ey "ould alway" u"e "un protetion

ream7 lon;"leeed "irt7 at or umrella wen it will run in te daytime.

Worker" in te o55ie "ould al"o e proteted aain"t "unlit 5rom te

window. 'n addition7 patient" "ould al"o aoid "mokin !ES. Beau"e

in5etion" o5ten our in patient" wit !ES7 te patient "ould e reminded

wen eperienin uneplained 5eer7 e"peially in patient" tat reeied i

do"e" o5 ortio"teroid"7 ytotoi dru"7 patient" wit kidney 5ailure7 eart

ale eetation7 uler" in te "kin and muo"a. ropylati antiioti" "ould

e on"idered in patient" wo will undero te proedure !ES enitourinary7

toot etration and oter ina"ie proedure".

Birt ontrol i" ery important in patient" wit !ES7 e"peially patient" wit

nepriti"7 or patient" reeiin dru" tat are ontraindiated 5or prenany7 5or

eample antimalarial or ylopo"pamide. renany an al"o trier aute

eaeration" !ES and a" it" own ri"k to te 5etu". -ere5ore7 monitorin o5

di"ea"e atiity durin prenany "ould e more "trinent.



Be5ore te !ES patient" ien treatment7 mu"t deide 5ir"t weter te patient

i" la""i5ied a" reDuirin on"eratie terapy7 or immuno"uppre""ie

are""ie. 'n eneral7 patient" wit !ES are not li5e treatenin and i" not

a""oiated wit oran damae7 an e treated on"eratiely. Wen te di"ea"e

i" li5e treatenin and on ma6or oran"7 it i" on"idered are""ie terapy tat

inlude" i;do"e ortio"teroid" and oter immuno"uppre""ant".

26<61 TERAPY ;ONSERVATIF

26<6161 Ar"#ri"i> ar"#ra&(ia an$ Mia&(ia

Artriti"7 artralia7 and &yalia i" a ommon omplaint in patient"

wit !ES. 'n lit o5 omplaint" tat an e ien "imple anale"i"

or non"teroidal antiin5lammatory dru". Wi mu"t e on"idered in

te u"e o5 te"e dru" i" teir "ide e55et" in order not to araate teeneral "tate o5 te patient. Ader"e e55et" on te a"trointe"tinal

"y"tem7 lier and kidney" mu"t e on"idered7 5or eample y

reularly ekin "erum reatinine. '5 anale"i" and non"teroidal

anti;in5lammatory dru" do not ie a ood re"pon"e7 an e

on"idered te proi"ion o5 antimalarial dru"7 "u a"

ydroyloroDuine 400 m Q day. '5 witin # mont"7 ti" dru do not

ie a ood e55et7 "ould e immediately "topped. )iin more tan

3 mont" loroDuine or ydroyloroDuine 5or more tan # mont"

reDuire optalmoloi ealuation7 eau"e te"e dru" ae toie55et" on te retina.

'n "ome patient" wo do not "ow adeDuate re"pon"e to anale"i" or

non;"teroidal anti;in5lammatory dru" or antimalarial dru"7 an e

on"idered a low;do"e ortio"teroid"7 wit a do"e o5 no more tan 1$

m7 eery mornin. !ow;do"e metotreate <(.$;1$ m Q week=7 may

al"o e on"idered to addre"" artriti" in patient" wit !ES.

26<6162 L%% 7%"ane%

Approimately (0M o5 patient" will eperiene poto"en"itiity !ES.

Aute eaeration" !ES an ari"e wen patient" are epo"ed to

ultraiolet lit7 in5rared lit7 eat and "ometime" al"o te

5luore"ene lit. atient" wit poto"en"itiity "ould proide

protetion aain"t epo"ure to N;ray" y u"in protetie lotin7

la"" window" darkened7 aoid diret epo"ure and u"e "un"reen.

&o"t "un"reen" topial 5orm o5 a ream7 oil7 lotion or el ontainin



ABA and it" e"ter"7 enopenone7 "aliyli and innami tat an

a"or ultraiolet A and B. -i" Sun"reen "ould alway" e reu"ed

a5ter atin or "weatin. !oal luoortioid"7 "u a" ream7

ointment or in6etion an e on"idered in lupu" dermatiti". Seletion

o5 topial preparation" "ould e are5ul7 eau"e topial

luoortioid"7 e"peially to"e tat are di5lorina"i an au"e "kin

atropy7 depimentation7 teleaniekta"i" and 5raility. %aial

reommended te u"e o5 a low power loal "teroid preparation" and

not di5lorina"i7 "u a" ydroorti"one. -o "kin te ody and arm

"trent topial "teroid" may e u"ed are7 5or eample7 etameta"one

alerate and triaminolone aetonide. %or ypertropi le"ion"7 5or

eample in te area o5 palmar and plantar pedi"7 an u"e i;"trent

topial luoortioid"7 e7 etameta"one dipropionate. -e u"e o5

i;"trent ream luoortioid" "ould e re"trited 5or 2 week"7and ten replaed wit a manitude lower.

Antimalarial dru" are ery ood to deal wit utaneou" lupu"7

"uaute utaneou" lupu" ood7 and di"oid lupu". Antimalarial ae

"un"lokin e55et"7 anti;in5lammatory and imuno"upre"an.ada

patient" wo are re"i"tant to antimalarial7 an e on"idered

pemerikan "y"temi luoortioid". ap"one admini"tration an e

on"idered in patient" wit di"oid lupu"7 a"uliti" and erula !E

le"ion". -oi e55et" o5 ti" dru on te ematopoieti "y"tem i"

metemoloinemia7 "ul5emoloinemia7 and emolyti anemia7

wi "ometime" eaerate te !ES in te "kin ra".

26<6163 Fa"i(%e an$ co/&ain" i"e/ic

%atiue i" a ommon omplaint in patient" wit !ES7 a" well a"

weit lo"" and 5eer. %atiue an al"o ari"e due to luoortioid

terapy7 werea" weit lo"" and 5eer an al"o e au"ed y rantin

Duinakrin. y"iian" "ould e "ympaty in addre""in ti" prolem.

K5ten it doe" not reDuire "pei5i treatment7 "imply add a reak and "et

workin our". 'n "eere irum"tane" tat may indiate an inrea"e

in di"ea"e atiity !ES and admini"tration o5 "y"temi luoortioid"

may e on"idered.

26<616! Seroi"i

Ce"t pain and adominal pain in patient" wit !ES an e a "in o5

"ero"iti". 'n "ome patient"7 ti" ondition an e treated wit

"aliylate"7 non;"teroidal anti;in5lammatory dru"7 antimalarial"7 or

low;do"e luoortioid" <1$ m Q day=. 'n "eere irum"tane"7

"ould e ien "y"temi luoortioid" to ontrol te di"ea"e.



AGGRESSIVE TREATMENT

26<6261 ;or"i7o"eroi$

Are""ie terapy tat ean wit te admini"tration o5 i;do"eluoortioid" "ould e "tarted immediately in a"e o5 "eriou"

mani5e"tation o5 te !ES and li5e treatenin7 "u a" a"uliti"7

utaneou" lupu" eay7 poliartriti"7 poli"ero"iti"7 myoarditi" lupu"

pneumoniti"7 lomerulonepriti" <proli5eratie 5orm"=7 emolyti

anemia7 tromoytopenia7 rain "yndrome orani7 "eere onitie

de5et"7 myelopaty7 periperal neuropaty and lupu" ri"i" <i

5eer7 pro"tration=.

)luoortioid do"e i" ery important to note tan oter type" o5

luoortioid" to e proided. oweer7 admini"tration o5 luoortioid" "u a" deameta"one lon e55et7 "ould e aoided.

redni"one more pre5erred7 eau"e it i" more ea"ily ad6u"t te do"e.

)iin oral luoortioid"7 "ould e ien in a "inle do"e in te

mornin. 'n !ES minor mani5e"tation"7 "u a" artriti"7 "ero"iti" and

on"titutional "ymptom"7 an e ien predni"one 0.$ m Q k Q day7

wile te ma6or and "eriou" mani5e"tation an e ien predni"one 1;

1.$ m Q k Q day. Bolu" admini"tration o5 intraenou"

metylpredni"olone 1 or 1$ m Q k 5or 3 day" an e on"idered a"

a "u"titute 5or i;do"e oral luoortioid"7 5ollowed y oral predni"one 1;1.$ m Q k Q day.

-erapeuti re"pon"e an e "een a" early a" po""ile7 ut an al"o in

a lon time7 "u a" #;10 week". A5ter admini"tration o5 i;do"e

luoortioid" 5or # week"7 ten it "ould "tart do derea"e your do"e

radually7 "tartin wit $;10M eery week i5 not ari"e aute

eaeration". A5ter te do"e o5 predni"one at 30 m Q day7 te do"e

redution made 2.$ m Q week7 and a5ter do"e predni"one 10;1$ m Q

day7 a do"e redution wa" per5ormed 1 m Q week. Wen ari"e aute

eaeration7 predni"one do"e i" inrea"ed up to te e55etie do"e7ten try lowered ak.

'5 witin 4 week" a5ter admini"tration o5 i do"e" o5 luoortioid"

"owed no notieale improement7 on"idered to proide oter

immuno"uppre""ant" or oter are""ie terapy.

26<6262 Si7&ooa/i$

'ndiation" o5 ylopo"pamide on te !ES:

1. atient" !ES reDuirin i;do"e "teroid" <"teroid "parin aent=.



2. atient" wit !ES are ontraindiated 5or i;do"e "teroid".

3. atient" wo relap"e !ES a" een treated wit "teroid" lon;term

or repeated.

4. -e initial di55u"e lomerulonepriti".

$. !ES wit tromoytopenia are re"i"tant to "teroid".

#. -e deline in lomerular 5iltration rate or an inrea"e in "erum

reatinine witout adnya oter etrarenal 5ator".

(. !ES wit entral nerou" "y"tem mani5e"tation".

Bolu" intraenou" ylopo"pamide 0.$;1 Q m2 in 1$0 ml o5 0.9M

?aCl 5or #0 minute" 5ollowed y admini"tration o5 2;3 liter" o5 5luid Q

24 our" a5ter admini"tration o5 te dru7 widely u"ed in te treatment

o5 !ES. Cylopo"pamide wa" ien 5or # mont" wit interal" o5 1

mont7 ten eery 3 mont" 5or 2 year". urin te admini"tration o5

ylopo"pamide7 te "teroid do"e i" radually lowered wit reard

lupu" atiity. 'n patient" wit derea"ed renal 5untion up to $0M7

ylopo"pamide do"e wa" lowered to $00;($0 m Q m2.

A5ter admini"tration o5 ylopo"pamide7 lood leukoyte ount""ould e monitored. Wen te numer o5 leukoyte" 17$00 Q ml7 ten

te net ylopo"pamide do"e redued y 2$M. %ailure "uppre""

leukoyte ount to 4000 Q ml "owed a do"e ylopo"pamide

inadeDuate "o tat te do"ae "ould e inrea"ed 10M in te net

admini"tration.

Cylopo"pamide toiity inlude nau"ea and omitin7 alopeia7

emorrai y"titi"7 "kin malinanie"7 oarian 5untion "uppre""ion

and aoo"permia.

26<6263 A?a"iorin

Aatioprine i" a purine analo tat an e u"ed a" an alternatie to

ylopo"pamide at a do"e o5 1;3 m Q k Q day and ien orally.

-e"e dru" an e ien 5or #;12 mont" in patient" !ESV a5ter te

di"ea"e an e ontrolled and kept to a minimum do"e o5 "teroid"

already7 ten te do"e o5 aatioprine may al"o e lowered "lowly and

"topped a5ter illne"" really well ontrolled.



-e toiity o5 aatioprine inlude "uppre""ion "y"tem emopoetik7

eleated lier enyme" and trier malinany.

26<626! Si7&oorin

Kter immuno"uppre""ant" wi an e u"ed 5or te treatment o5

!ES i" Cylo"porine low do"e <3;# m Q k Q day=. -e"e dru" an e

u"ed on ot te !ES witout renal mani5e"tation" and wit

memranou" nepropaty. urin te admini"tration "ould note te

patient" lood pre""ure and lood reatinine leel". '5 leel" o5 lood

reatinine inrea"ed 20M 5rom lood reatinine leel" e5ore

admini"tration o5 ylo"porine7 and te do"e "ould e lowered.

26<6265 Moe"i&@/:co#eno&a"e 'MMF,

&&% may redue te atiity and mortality o5 patient" wit !ES. 'n

lupu" nepriti"7 &&% a" a omparale e55et wit ylopo"pamide

in term" o5 remi""ion7 reurrene and ri"k o5 in5etion. &&% an

maintain remi""ion o5 lupu" nepriti" leel omparale wit te lon;

term ylopo"pamide. &&% wa" not a""oiated wit one marrow

"uppre""ion7 or amenorrea. &&% do"e i" $00 ; 1$00 m7 2 time" per

day.

26<626- Ri"%8i/a)

+ituima i" a monolonal anti;C20 antiody7 wi an e u"ed in

te treatment o5 "y"temi autoimmune di"ea"e"7 inludin !ES.

+ituima do"e i" 1 ram7 2 time" iin te di"tane o5 2 week"7 and

an e repeated eery # mont".

26<626. I//%no(&o)%&in G IV2!

)iin intraenou" immunoloulin i" al"o u"e5ul to oerome

tromoytopenia in !ES7 at a do"e o5 300;400 m Q k Q day7admini"tered 5or $ on"eutie day"7 5ollowed y maintenane do"e"

eery mont to preent reurrene. A"olute ontraindiation to te

admini"tration o5 immunoloulin 'A de5iient patient" wo

"ometime" 5ound in patient" wit !ES.

26= Dieae Pro(noi LES2

-e prono"i" depend" on wi oran" are inoled. '5 te ital oran"7

mortality i" ery i. &ortality in patient" wit !ES a" delined oer te

pa"t 20 year". rior to 19$$7 te "urial rate at $ year" in S!E i" le"" tan



$0M. -oday7 te "urial rate in te la"t 10 year" on aerae eeed 90M and

te "urial rate at 1$ year" wa" aout 80M. Surial rate in te la"t 10 year" in

A"ia and A5ria i" "ini5iantly lower7 ranin 5rom #0;(0M. +edution in

mortality a""oiated wit te !ES an e a""oiated wit te deteted early

diano"i"7 improement" in te treatment o5 di"ea"e" o5 te !ES7 and prore""

in te eneral medial are

Po&:/:a&(ia r#e%/a"ic

Deini"ion

-i" di"ea"e i" a "yndrome on"i"tin o5 pain and "ti55ne""7 e"peially on te

proimal lim mu"le"7 nek7 "oulder7 and peli". E"peially aout middle ae

or older. &ay ari"e a" a prodromal or mani5e"tation" o5 ariou" di"ea"e"7 may

al"o our witout au"in di"ea"e7 polymyalia reumati alled primary.

C&inica& Manie"a"ion

-e main "ymptom" are pain and "ti55ne"" o5 te nek7 "oulder"7 upper arm"7

wai"t7 ip"7 and lower ak. Sti55ne"" partiularly di"turin "leep and re"t.-ere i" no mu"le 5atiue. &ay e aompanied y eadae7 anoreia7

malai"e7 5eer7 and a lot o5 "weat7 o5ten aompanied y depre""ion. Kn py"ial

eamination 5ound no anormality typial.

S%or"in( In+e"i(a"ion

%rom te re"ult" o5 laoratory te"t"7 otained normokrom normoyti anemia

mild7 mild leukoyto"i"7 eleated ES+ and reumati 5ator were neatie. Kn

radioloial eamination7 poto" enerally normal 6oint"7 a" well a"

eletromyorapy.

Mana(e/en"

olymyalia reumati witout arteriti" "owed rapid and dramati re"pon"e

witin 1;$ day" wit a "mall do"e o5 predni"one 10;1$ m daily. '5 tere are

"ymptom" tat may e au"ed ranial arteriti"7 it i" reommended admini"tration

o5 predni"one #00 m Q day a" te initial do"e7 5ollowed y temporal artery

iop"y 1;2 week" terea5ter. redni"one wa" ien 5or 1;2 mont" e5ore

taperin o55.

Pro(noi



ain in te mu"le" wit ood moement enerally re"ole" it"el5 witin "i

mont"7 ut may e up to 2;3 year".

2.

Autoimmune di"order i" a mal5untion o5 te immune "y"tem wi make" te ody

attak" it" own ti""ue". @eep te ody" immune "y"tem aain"t wat ini"iility a"

5orein or danerou" "u"tane". Su material" inludin miroorani"m"7 para"ite"

<"u a" worm"=7 aner ell"7 and een tran"plantation o5 oran" and ti""ue".

&aterial" tat an "timulate te immune re"pon"e are alled antien". Antien" are

moleule" tat may e ontained in te ell or on te "ur5ae o5 ell" <"u a"

ateria7 iru"e" or aner ell"=. Some antien"7 "u a" pollen or 5ood moleule"7 i"

on teir own.

Cell" een in people wo ae teir own network" an ae te antien. But7 u"ually7

te immune "y"tem reat" only to antien" o5 5orein material" or danerou"7 not to

antien" o5 people wo ae a network 5or one"el5. oweer7 "ometime" te immune"y"tem i" damaed7 tran"late te ody" own ti""ue" a" 5orein and produe"

antiodie" <alled autoantiodie"= or immune ell" to taret and attak te ody"

own ti""ue".

-i" re"pon"e i" alled an autoimmune reation. 't re"ult" in in5lammation and ti""ue

damae. Su e55et" may e an autoimmune di"order7 ut "ome people produe a

"mall amount o5 autoantiodie" tat autoimmune di"order doe" not our.

Some ommon autoimmune di"order" "u a" reumatoid artriti"7 "y"temi lupu"

erytemato"u" <lupu"=7 and a"uliti"7 amon oter". Additional di"ea"e elieed to

e a""oiated wit "u autoimmune lomerulonepriti"7 Addi"on" di"ea"e7

onnetie ti""ue di"ea"e miture7 S6oren" "yndrome7 prore""ie "y"temi

"lero"i"7 and "ome a"e" o5 in5ertility.

3.



&ani5e"tation o5 :

S"e+en on#onB S:n$ro/e

Steen";,on"on "yndrome u"ually ein" wit a 5eer7 "ore troat7 5atiue7 and pain in te 6oint". &o"t patient" are mi"diano"ed and treated wit antoiotik.

ler" and le"ion" <"kin li"ter"= ein to appear on te muou" memrane7

almo"t alway" in te oral Q mout and al"o in te enital and anal area". -e"e

"ymptom" are ery pain5ul and an lead to derea"ed appetite and drinkin 5or

to"e wo eperiene "ymptom" in te mout area. Eye on6untiiti" our"

around 30M in ildren wit ti" "yndrome. !e"ion" ra" Q li"ter" appeared aout

an in o5 "kin on te 5ae7 arm" and le" a" well a" palm"7 ut it u"ually doe" not

appear on te "alp.

enoc# Sc#on&ein P%r%raS in eneral aute on"et and "udden. -e main linial 5eature" on"i"t o5 5our

oran" inoled. %ir"t on te "kin were tere i" leedin "litly eleated "kin i5

toued <palpale purpura= our" in 9$;100M o5 a"e" wi mainly our" on

part" o5 te ody are dependent or under pre""ure a" lower le"7 uttok"7 ody

and and". Bleedin in te 5orm o5 pate" o5 rit red or dark red or lui" tat

an e 5u"ed. -i" erdaraam will enerally di"appear witin a 5ew day" to

"eeral mont". !e"" tan 10M o5 a"e" an e repeated and may "ettle a 5ew

year". -i" leedin an e aompanied y "wellin <edema=. 2 to te inoled

oran i" te a"tro;inte"tinal trat. Symptom" tat appear on ti" oran i" a reatadominal pain <adominal oli=7 nau"ea and omitin to our a"trointe"tinal

leedin <intu""u"eption=7 wi u"ually appear 1 week a5ter te appearane o5

te "kin leedin. ,oint" are te oran" inoled to 3. Cild "uddenly an not

walk7 pain5ul 6oint" <artralia= or until te "wellin o5 te 6oint"7 pain7 redne""

and wen toued 5eel" ot <atriti"=. &ore a55eted 6oint" knee or ankle 6oint".

-e kidney" to 4 are inoled. %a"ter rowin in adult". Symptom" may inlude

ematuria <urine reddi"=7 roteinuri. '5 "ymptom" o5 miro"opi ematuria only

a kidney anormalitie" mild lomerulonepriti" ut in a"e o5 rapidly prore""ie

lomerulonepriti" will au"e roni yperten"ion an een enter into end;"tae

kidney di"ea"e.

%+eni& Ar"#eri"i C#ronic

'" te main linial "ymptom" "een o6etiely. &arked y one o5 te "ymptom"

o5 "wellin or 6oint e55u"ion7 or at lea"t 2 o5 te 3 "ymptom" o5 in5lammation are

limited moement7 pain wen atuated and eat. ain or pain i" u"ually not "o

prominent. 'n youn ildren7 te more oiou" i" te 6oint "ti55ne"" in moement7

e"peially in te mornin <mornin "ti55ne""= .9

,+A "utype" depend" on te "y"temi "ymptom" o5 te di"ea"e and te numer

o5 a55eted 6oint" in te 5ir"t # mont" our"e o5 te di"ea"e. Son "aid to ae on"et



,+A ; "y"temi on"et di"ea"e wen aompanied y i 5eer oin <at lea"t

40 C= 5or up to 2 week" and <u"ually= y a ra" tat Duikly di"appeared at te

eit o5 5eer witout a55eted te numer o5 a55eted 6oint" durin te 5ir"t #

mont". 'n ,+A ,+A7 aout le"" tan $ 6oint" in te 5ir"t # mont"7 polyartiular

di"ea"e inole" 5ie or more 6oint". Ea "utype o5 di"ea"e7 altou it i" merely

de"riptie7 "owin te our"e o5 di"ea"e7 ompliation"7 and prono"i" are

di55erent.

4.

Compliation o5 :

1. Sy"temi Eritematou" !upu" <SE!=

Seere lupu" an a55et oran damae7 amon oter":

• @idney <lupu" nepriti"= wit renal 5ailure.

• -e re"ult o5 in5lammation o5 te eart linin o5 te eart <periarditi"=

and i"emi eart di"ea"e.

• !un i" in5lammation o5 te linin o5 te lun <pleuri"y= and

pneumooal <pneumonia=.

• ?erou" "y"tem and p"yiatri 5orm o5 "eiure"7 paraly"i"7 "troke7

depre""ion and p"yo"i"

• Eye"7 amon oter": atarat

• 'n prenant women an our mi"arriae7 premature irt and

neonatal lupu"

• Blood "y"tem di"order" "u a" anemia7 lak o5 wite lood ell"

<leukopenia= and le"" lood lottin ell" <tromoytopenia=.

2. Slenoderma

-e prono"i" i" wor"e 5or i55u"e Cutaneou" di"ea"e7 partiularly in older

ae7 and 5or men. -e mo"t ommon deat 5rom ompliation" o5 lun7lier and kidney. 'n i55u"e Cutaneou" di"ea"e7 $;year "urial wa" (0M7

10;year "urial o5 $$M.

3. oliartriti" ?odu"a

An immune omple di"ea"e mu"ular arterie" and arteriole". -e di"ea"e

i" rarely aout lun and unknown etioloy. Symptom" tat an e 5ound

are: artralia7 myalia7 periperal nere di"order"7 redne""7 nodule" on te

"kin7 adominal pain7 yperten"ion7 and eart 5ailure <%=. 'n "ome a"e"



tere i" a owel per5oration and in"tu"u"ep"i ileoilel au"ed y a"uliti"

tat au"e" te inte"tinal wall edema and "umuo"al leedin.

4. /a"uliti" !upu"

Kran inolement in addition to "kin ra"e" ery rare7 ut an e "eere.

'n5lammation o5 te kidney" and een more rarely in6ured lier7 lun7 eart

and rain ae ourred in patient" wit yper"en"itiity a"uliti".

@idney in5lammation i" u"ually mild.

Compliation" may inlude permanent damae to te lood e""el" or "kin

wit "arrin.

SOURCE



. ermono Baman7 Sutarno7 ra"ena.')7 dkk. Buku Ajar Hemato

Onkolo! Anak "K UI#'katan okter 'ndone"ia 2012

Sudoyo Aru.W7 Setiyoadi Baman7 Alwi 'dru"7dkk. Ilmu $en%ak!t &alam '!l!d

Ed!! *#

laporan sk4 klp 13

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