Komplikasi Penderita Sistemik Sklerosis di RSUP Dr.

Kariadi Semarang
?aya S SuhasLlnah 8ahmayanLl P* 8anLar S* u Sudarsono* SuyanLo P*
*SLaff sub 8aglan 8eumaLologl 8aglan enyaklL ualam lk unlverslLas ulponegoro/8Su ur karladl
A8S1kAk
Latar 8e|akang
Sklerosls slsLemlk aLau skleroderma adalah penyaklL [arlngan lkaL yang [arang dlLandal oleh
flbrosls pada kullL pembuluh darah dan organorgan vlsceral revalensl slsLemlk sklerosls
dlperklrakan anLara 4 dan 233 kasus per [uLa orang lakLorfakLor seperLl usla [enls kelamln
laLar belakang geneLlk dan paparan llngkungan dapaL mempengaruhl kerenLanan vlrus
seperLl Puman arvovlrus 819 cyLomegalovlrus vlrus LpsLeln8arr dan herpes slmplex
vlrus Llpe 1 dlduga LerllbaL dalam Ler[adlnya sclerosls slsLemlk WanlLa Lerkena 3 kall leblh
serlng lakllakl dan usla onseL adalah khas dl 40an dan 30an
enellLlan yang dllakukan oleh 1homas dan vlrglnla dl lLLsburgh Lerhadap 933 paslen
dengan skleroderma dlfus Lampak kompllkasl Lerhadap gln[al 19 [anLung 13 paru 16
saluran Cl 8 dan keLerllbaLan kullL pada 24
Metode ene||t|an
uesaln penellLlan ueskrlpLlf dengan sampel semua penderlLa slsLemlk sklerosls yang berobaL
dl 8Suk darl Lahun 2006 2011 1u[uan penellLlan unLuk memperoleh daLa paslen dan
mengeLahul karakLerlsLlk penderlLa slsLemlk sklerosls Sampel ber[umlah 9 penderlLa
slsLemlk sklerosls
nas|| ene||t|an
rofll penderlLa slsLemlk sklerosls yang berobaL rawaL lnap dl 8Su ur karladl 100 [enls
kelamln perempuan 8erdasarkan usla penderlLa 22 berusla 2130 Lahun 33 usla 3140
Lahun 22 usla 4130 Lahun 11 usla 3160 Lahun dan 11 usla 6170 Lahun kompllkasl
muskuloskleleLal 100 dengan manlfesLasl pollarLrlLls paru 66 dengan gambaran flbrosls
paru 77 dengan sklerodakLlll dan gangguan gasLrolnLesLlnal 22 dengan manlfesLasl
dlsmoLlllLas esophagus
kes|mpu|an
rofll penderlLa sklerosls slsLemlk yang berobaL rawaL lnap dl 8Su ur karladl domlnan
ber[enls kelamln perempuan usla Lerbanyak anLara 3140 Lahun dan dengan manlfesLasl
kllnls Lerbanyak pollarLrlLls

This study demonstrates that severe
organ invoIvement in SSc patients with diffuse scIeroderma
most often occurs earIy in the course of the
disease. SurvivaI for patients with severe organ invoIvement
is markedIy reduced. Patients shouId therefore be
monitored very cIoseIy during the first 3 years of disease
for signs and symptoms that may signaI the subsequent
deveIopment of severe organ damage. PotentiaI diseasemodifying
therapies must be initiated earIy to modify
the naturaI history of SSc and to improve survivaI.
Patients who survive the first few years without deveIoping
severe organ invoIvement are Iess IikeIy to deveIop
such Iife-threatening invoIvement Iater in the disease
course.


Of the 953 patients with diffuse scIeroderma, kidney invoIvement deveIoped in 177
(19%), heart invoIvement in 143 (15%), Iung invoIvement in 151 (16%), GI tract
invoIvement in 74 (8%), and skin invoIvement in 233 (24%). Severe skin and kidney
invoIvement occurred during the first 3 years in 70% of those who ever deveIoped
these probIems throughout a mean of 10 years of foIIowup. Severe heart, Iung, and GI
tract invoIvement deveIoped during the first 3 years in 45-55% of those who were ever
affected. The survivaI of patients with severe organ invoIvement was poor. The 9-year
cumuIative survivaI rate of aII patients with severe organ invoIvement was 38%,
compared with 72% in patients without such invoIvement (! 0.0001).
VRGNA D. STEEN and THOMAS A. MEDSGER, JR.