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Karakteristik purpura henoch-schonlein pada anak di RSCM periode Januari 2009 - Desember 2012 = Characteristics of children with henoch-schonlein purpura in RSCM on January 2009 - Desember 2012

Latar 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.
Kata kunci : purpura Henoch-Schӧnlein, anak, karakteristik, Indonesia


Background : 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 1 2009 to December 31 st 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.
Keywords : Henoch-Schӧnlein purpura, children, characteristics, Indonesia