ABSTRAKLatar belakang : Purpura Henoch-Schӧnlein (PHS) merupakan sindrom klinis
yang disebabkan vaskulitis akut sistemik pada pembuluh darah kecil yang paling
sering pada anak. Manifestasi klinis PHS sering melibatkan berbagai organ seperti
kulit, sendi, gastrointestinal, dan ginjal. Rekurensi terjadi pada hampir 50 % kasus
dan memengaruhi prognosis PHS. Sejauh ini belum ada publikasi penelitian PHS
yang meliputi manifestasi klinis, laboratorium, serta rekurensi di Indonesia.
Tujuan : Mengetahui manifestasi klinis, laboratorium serta rekurensi PHS anak di
Indonesia.
Metode : Penelitian deskriptif retrospektif. Data diperoleh dari rekam medis
pasien anak berusia 0-18 tahun dengan diagnosis PHS selama periode 1 Januari
2009 hingga 31 Desember 2012 di Rumah Sakit Cipto Mangunkusumo, Jakarta.
Hasil : Terdapat 71 kasus PHS dengan rentang usia 2 sampai 16 tahun dan usia
tersering pada kelompok 6-8 tahun. Proporsi anak wanita lebih tinggi dibanding
lelaki dengan rasio 1,2:1. Semua pasien mengalami purpura palpabel dan
manifestasi tersering lainnya adalah gangguan gastointestinal (79 %), artritis atau
artralgia (68 %), dan keterlibatan ginjal (41 %), sedangkan yang jarang adalah
gangguan neurologis (1 %), dan edema skrotum (4 %). Riwayat infeksi yang
mendahului gejala PHS didapatkan pada 56 % kasus. Peningkatan laju endap
darah (88 %) dan trombositosis (60 %) merupakan kelainan laboratorium yang
paling sering ditemukan, diikuti dengan hematuria (41 %), leukositosis (32 %),
dan anemia (31 %). Penurunan fungsi ginjal ditemukan pada 6/42 kasus.
Perbaikan gejala klinis terlihat dalam waktu kurang dari 4 minggu untuk
manifestasi kulit, gastrointestinal, dan persendian. Sebanyak 18/24 subjek dengan
hematuria mengalami perbaikan dalam waktu 6 bulan. Penurunan fungsi ginjal
menetap tidak ditemukan dalam penelitian ini. Rekurensi didapatkan pada 5/57
subjek yang memiliki data pemantauan.
Simpulan : Manifestasi klinis tersering pada PHS adalah purpura palpabel,
gangguan gastrointestinal, artritis atau artralgia, dan keterlibatan ginjal, sedangkan
yang jarang adalah gangguan neurologis dan edema skrotum. Pemeriksaan darah
perifer lengkap dan urinalisis sebaiknya dilakukan pada semua pasien PHS untuk
mendukung diagnosis dan menilai keterlibatan ginjal. Pada semua pasien PHS
sebaiknya dilakukan pemantauan minimal selama 6 bulan untuk menilai
keterlibatan ginjal yang mungkin timbul terlambat serta rekurensi
ABSTRACTBackground : Henoch-Schӧnlein purpura (HSP) is a clinical syndrome which
caused by systemic acute vasculitis in small vessel. Henoch-Schӧnlein purpura is
the most common etiology of vasculitis in children. Clinical manifestations
usually involved several organs, such as skin, joint, gastrointestinal, and kidney.
Recurrency occured in almost 50 % cases, and lead to poor prognosis. Up to now,
there was no publications of HSP study in Indonesia regarding in clinical profiles,
laboratory, and recurrency.
Objective : To investigate the clinical characteristics, laboratory, and recurrency
of HSP in Indonesian’s children.
Method : A retrospective descriptive study was conducted from medical records
of children up to 18 years, in Cipto Mangunkusumo Hospital (CMH). Our
participants were children diagnosed as having HSP from January 1st 2009 to
December 31st 2012.
Results : There were 71 cases of HSP, with the range of age from 2 years old to
16 years old. Mostly subjects were at group age between 6 and 8 years old. Girl
was commonly affected compared to boy (1.2:1). All patients had palpable
purpura, other clinical symptoms that usually occured were gastrointestinal
(79 %), arthritis or arthralgia (68 %), and kidney disorder (41 %). Neurologic
symptoms (1 %) and scrotal edema (4 %) were the least found. 56 % of HSP
patient was preceeded by infection history. Laboratory results that commonly
found were increasing of ESR (88 %), thrombocytosis (60 %), hematuria (41 %),
and anemia (31 %), respectively. Kidney function impairment was occured in 6/42
cases. Clinical symptoms improvement had shown in less than 4 weeks for skin,
gastrointestinal, and joint disorder. Eighteen of twenty four subjects with
hematuria had recovery within 6 months. There were no cases of persistent kidney
function impairment. Recurrency occured in 5/57 subjects.
Conclusion : Clinical manifestations that commonly found in HSP patients were
palpable purpura, gastrointestinal disorder, arthritis or arthralgia, and kidney
involvements. Neurological disorder and scrotal edema were less found. Routine
blood and urine examination should be done in all HSP patients to confirm the
diagnosis and evaluate kidney involvement. In all HSP patients, we suggest to do
follow up on evaluating late kidney involvement and recurrency minimally in 6
months period.