Sklerosis Sistemik Difus - Wachyoe Hadi Saputra (Universitas Syiah Kuala, 2010)
Abstrak. Sklerosis sistemik (sklerodenna) adalah penyakit jaringan ikat yang tidak diketahui penyebabnya yang ditandai oleh fibrosis kulit dan organ visceral serta kelainan mikrovaskular. Prevalensi pasien ini relatif rendah dengan 50-300 kasus setiap I juta penduduk dan insidensi 2,3-22,8 kasus setiap 1 juta penduduk per tahun, serta resiko pada wanita lebih tinggi dibanding pria dengan rasio 3-4:1. Diagnosis ditegakkan berdasarkan gambaran klinik dan pemeriksaan penunjang. Penatalaksanaan pada pasien hanya mengurangi atau menghilangkan keluhan dim memperbaiki fungsi organ tetapi tidak dapat menyembuhkan pasien. Angka harapan hidup rata-rata pada pasien skJerosis sistemik difus 70% dalam 5 tahun dan 55% dalam 10 tahun, Dilaporkan sebuah kasus sklerosis sitemik difus pada seorang wanita, usia 34 tahun. Diagnosa ditegakkan berdasarkan kriteria ARA (penebalan, penegangan dan pengerasan kulit di seluruh ekstemitas, muka, leher, dan batang tubuh atau punggung serta h.ilangnya substansi jari), anti Sci-70, photo manus, biopsi kulit paha, gastroduodenoskopi dan ekokardiografi. Setanjutnya pasien dilakukan penatalaksanaan dengan vasodilator, imunosupresif, antifibrotik, dan penghambat pompa proton. (JKS 2010;2193-98) Kata kunci : Sklerosis sistemik difus, manifestasi klinik Abstract. Systemic sclerosis (scleroderma) is a connective tissue disease of the unknown causes that characterized by fibrosis of skin and visceral organs as well as micro vascular abnormalities. The prevalency of the patients is relatively low with 50-300 cases per 1 million of the population and the incidence of 2.3 to 22.8 cases per 1 million population per year; however, the risk on women is higher than men with a ratio of 3-4:1. The diagnose of this disease is based on clinical examinations and other supporting tests. The management of the patients is done just to reduce or eliminate the complaints and to improve organ functions but unfortunately can not cure the disease. The figure for average lifeexpectancy in patients with diffuse systemic sclerosis is about 70% in 5 years and 55% in 10 years. There was a case of systemic diffuse sclerosis disease on 34 year old woman. The diagnosis was based on the ARA criteria (thickening, tightening and hardening of skin around extremities, face, neck, and trunk or back and the Joss of fingers substances), anti-SCL-70, manus rontgen, thigh skin biopsies, gastroduodenoscopy and echocardiography. The doctor did a series of treatment on the patient such as vasodilators, immunosuppressive, antifibrotic, and proton pump inhibitors. (JKS 2010;2:93-98)Keywords: diffuse systemic sclerosis, clinical manifestations