臨 床 藥 物 治 療 學 Therapeutics of Clinical Drugs ( ) Pathergy test ( ) 4 4 表 一 Behcet's Disease 診 斷 1990 International Study Group 12 ( a n t e r i o r u

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1 Therapeutics of Clinical Drugs 臨 床 藥 物 治 療 學 貝 賽 特 氏 症 之 治 療 臺 北 市 立 聯 合 醫 院 陽 明 院 區 藥 劑 科 藥 師 吳 素 芳 楊 瑛 碧 摘 要 (Behcet's disease BD) HLA-B ( human leukocyte antigen) (susceptibility) 1990 (International Study Group for Behcet's Disease) Behcet's disease oral ulcer genital ulcer 壹 前 言 1 (ICD-9-CM 136.1) 1937 Hulusi Behcet 2 HLA-B ( HLA-B51 ) 貳 流 行 病 學 ( ) (Silk Road Disease) / 參 診 斷 1990 Behcet's Disease 28 2 Jun 藥 學 雜 誌 第 111 冊 105

2 臨 床 藥 物 治 療 學 Therapeutics of Clinical Drugs ( ) Pathergy test ( ) 4 4 表 一 Behcet's Disease 診 斷 1990 International Study Group 12 ( a n t e r i o r u v e i t i s ) (posterior uveitis) (cells in vitreous on slit lamp examination or retinal vasculitis) ( e r y t h e m a nodosum) (pseudofolliculitis) (papulopustular lesions) (acneiform nodules) Pathergy test Pathergy test mm ( ) 8 pathergy test Sweet's syndrome (pyoderma gangrenosum) 6 圖 一 注 射 部 位 Pathergy reaction 呈 陽 性 反 應 5,6 肆 臨 床 表 現 5 一 口 腔 潰 瘍 1. ( < 10 mm ) ( ) 7 2. (> 10 mm)( ) 7 3. ( ) 8 圖 二 小 的 潰 瘍 圖 三 大 的 潰 瘍 106 THE JOURNAL OF TAIWAN PHARMACY Vol.28 No.2 Jun

3 (posterior uveitis) (retinal vasculitis) 25% 圖 四 泡 疹 狀 潰 瘍 5 二 生 殖 器 潰 瘍 72-94% ( ) 7 BD Reiter's syndrome 圖 六 後 葡 萄 膜 炎 5,6 四 皮 膚 病 灶 80% ( ) 8 ( ) 8 BD 圖 五 陰 囊 表 皮 潰 瘍 5,6 三 眼 睛 病 灶 30-70% 10% ( ) 8 (anterior and posterior uveal tract) (anterior uveitis with hypopyon) 1/3 圖 七 結 節 性 紅 斑 圖 八 膿 疹 狀 的 病 灶 28 2 Jun 藥 學 雜 誌 第 111 冊 107

4 臨 床 藥 物 治 療 學 Therapeutics of Clinical Drugs 6 五 關 節 炎 2/3 六 胃 腸 道 疾 病 Crohn's disease pathergy test Crohn's disease BD 七 中 樞 神 經 5-10% (Parenchymal brain) ( 80%) 5-10% 伍 治 療 5,9 一 局 部 治 療 prednisolone (0.5%) triamcinolone ( %) fluocinolone ( % ) b e t a m e t a s o n e ( 0. 1 % ) corticosteroid chlorexidine (0.12%) tetracycline (250 mg 250 ml ) lidocaine (2-5%) clobetasol propionate 5,9 二 全 身 治 療 IV corticosteroid calcineurin inhibitors (immunosuppressants) 5,9,11 三 藥 物 ( ) Colchicine ( mg/day) 14 6 RCT (randomised controlled trial) colchicine1.5 mg/day BD (nodular lesions) 116 BD colchicine1-2 mg colchicine 11 ( ) Thalidomide ( mg/day) thalidomide ( ) Methotrexate (MTX)(5-20 mg/ week) 108 THE JOURNAL OF TAIWAN PHARMACY Vol.28 No.2 Jun

5 ( ) Azathioprine (1-2.5 mg/kg/day) 73 RCT azathioprine ( ) Cyclophosphamide cyclophosphamide 0.75 mg/m 2 cyclosporine ( ) Calcineurin inhibitors (cyclosporine tacrolimus) 1. Cyclosporine (2-5 mg/day) BD BD BD 2. Tacrolimus cyclosporine BD tacrolimus ( ) 1. Interferon-alph (IFNalph) T (T-lymphocytes) ( 1-4 ) RCT IFN-alph 2. Infliximab 3-5 mg 陸 預 後 25 BD 40 柒 總 結 參 考 資 料 : 1. ( ) 2. Eguia A, Villarroel M, Martínez-Conde R, Echebarría MA, Aguirre JM. Adamantiades-Behçet disease: An enigmatic process with oral manifestations. Med Oral Patol Oral Cir Bucal 2006; 11: E Chung YM, Lin YC, Tsai CC, et al. Behcet's disease with uveitis in Taiwan. J Chin Med Assoc 2008; 71(10): Al-Otaibi LM, Porter SR, Poate TWJ. Behcet's disease: A Review. J Dent Res 2005; 84(3): Marshall SE, Behcet's disease. Best Pract Res Clin Rheumatol 2004; 18(3): Sakane T, Takeno M, Suzuki N, Inaba G. Behcet's Disease. New England Journal of Medicine 1999; 341: M Önder,* MA Gürer, The multiple faces of Behcet's disease and its aetiological factors, 2001 European Academy of Dermatology and Venereology JEADV (2001) 15, Marjan Yousefi: Dermatologic Aspects of Behcet's Disease: Multimedia, Retrieved from emedicine clinical knowledge base, institutional edition, Last Updated: 28 2 Jun 藥 學 雜 誌 第 111 冊 109

6 臨 床 藥 物 治 療 學 Therapeutics of Clinical Drugs 2011 february 20, article/ media 9. Roberto Tunes, Mittermayer Santiago. Behcet's Syndrome: Literature Review Current Rheumatology Reviews, 2009; 5: American Behcet's Disease Association G Hatemi, A Silman, D Bang et al. Management of Behçet disease: a systematic literature review for the European League Against Rheumatism evidence-based recommendations for the management of Behçet's disease Ann Rheum Dis : Behcet s Disease Wu Shu-Fang, Yang Ying-Pi Pharmacy of Department, Taipei City Hospital Yang-Ming Branch Abstract The geographical distribution of Behcet's disease (BD), mainly spanning countries between the Mediterranean countries. It always attack large-small vessels including artery vein. It is a multisystemic inflammatory disease characterized by oral and genital aphthosis as well as inflammation of the eye, skin, joints, gastrointestinal tract, vasculature, and even the central nervous system. Its pathogenicity is still not sure, susceptibility to BD is strongly associated with the presence of the HLAB51 allele. The diagnosis is made on the basis of the criteria proposed by the International Study Group for BD in The choice of the treatment depends on the patient's clinical manifestations. There could not have the standard treatment for all the patient. 110 THE JOURNAL OF TAIWAN PHARMACY Vol.28 No.2 Jun