Granulomatosis Wegener dengan Antineutrophil Cytoplasmic Antibodies (ANCA) Negatif pada Laki-laki Usia 38 Tahun
DOI:
https://doi.org/10.56951/medicinus.v34i2.72Kata Kunci:
granulomatosis wegener, ANCA, autoimunAbstrak
Latar belakang : Granulomatosis Wegener atau granulomatosis dengan poliangiitis adalah vaskulitis nekrotikan sistemik yang menyerang pembuluh darah berukuran kecil dan sedang. Penyakit ini sering berkaitan dengan antineutrophil cytoplasmic antibodies (ANCA). Faktor pencetus terjadinya granulomatosis Wegener antara lain faktor lingkungan, infeksi, obat-obatan dan genetik. Manifestasi klinis granulomatosis Wegener dapat berupa gejala tidak spesifik seperti nyeri otot, artralgia, demam, anoreksia dan penurunan berat badan. Ujud kelainan kulit granulomatosis Wegener dapat berupa vaskulitis leukositoklastik, purpura, ulkus dan nodul subkutan. Granulomatosis Wegener juga dapat menyerang berbagai organ.
Kasus : Seorang laki-laki berusia 38 tahun datang dengan keluhan muncul bercak kemerahan dan kaku pada wajah, tangan dan kaki. Status dermatologi pada regio fasialis tampak adanya saddle nose dan pada ekstremitas superior dan inferior tampak purpura multipel diskret. Pada pemeriksaan ANCA pasien didapatkan hasil negatif. Pemeriksaan gambaran histopatologi pada lapisan epidermis menunjukkan proliferasi padat sel-sel limfosit, perivaskular infiltrat dan sedikit sel neutrofil. Pada lapisan dermis didapatkan granuloma-granuloma yang terdiri atas banyak histiosit epiteloid dan makrofag berbuih serta pembuluh darah yang rusak dengan infiltrasi neutrofil pada dinding serta ekstravasasi eritrosit.
Diskusi : Granulomatosis Wegener merupakan penyakit autoimun langka berupa inflamasi granulomatosis pada saluran pernapasan atas dan bawah serta vaskulitis sistemik yang berhubungan dengan ANCA. Gejala yang timbul pada granulomatosis Wegener berupa gejala ringan, namun dapat berkembang progresif dengan gambaran klinis yang lebih berat. Hasil pemeriksaan ANCA negatif tidak dapat langsung menyingkirkan diagnosis granulomatosis Wegener. Terapi awal granulomatosis Wegener berupa cyclophosphamide dan glucocorticoid yang diberikan selama 3-6 bulan. Pasien menunjukkan perbaikan klinis yang cukup bermakna dengan berkurangnya purpura pada seluruh tubuh setelah pemberian terapi selama lima minggu.
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