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難治性ベーチェット病15例26眼に対し,免疫抑制剤シクロスポリン(CYA)治療開始後1年以上経過観察した結果を報告する.CYA10mg/kg/日より開始し眼症状および副作用に応じて量を増減した.
眼発作頻度はCYA治療前1.1±0.8回/月であったが,CYA投与開始後,0.4±0.3回/月と有意に減少した(p<0.005).視力は改善または不変が26眼中22眼にみられた.眼外症状(口腔内アフタや皮膚症状)の発生頻度はCYA治療前後で有意差はなかった.
眼発作とCYA最低血清中濃度および臨床免疫検査とに相関はなく,CYA有効性の指標はみいだせなかった.
副作用としては腎機能低下が全例にみられ,肝機能ではGOTまたはGPTが正常上限をこえた例が9例みられたが投与量を漸減するにつれて改善した.CYA投与中神経ベーチェット病2例,腸管ベーチェット病2例がみられ,今後十分な注意を要する.悪性腫瘍の発生は現在までのところみられない.
We treated 15 cases with severe Behçet disease with systemic cyclosporin A for its immunosuppressive effects. The ages of patients ranged from 26 to 56 years, including 14 males and 1 female. The cases were fol-lowed up for 12 to 27 months. All the cases had failed to respond to prior treatments with systemic colchicine and/or immunosuppressants such as cyclophosphamide. The initial dose of cyclosporin A was set at 10 mg/kg/ day, to be later modified according to the clinical symptoms and side effects.
The frequency of acute ocular attacks was signifi-cantly reduced after initiation of treatment. The treat-ment could be terminated because of very favorable response in 2 cases. The visual acuity remained or improved during the course of observation in 22 out of 26 affected eyes. Formation of lymphoblasts was signifi-cantly suppressed during the treatment. There was no relationship between the ocular attacks and laboratory findings concerning the immunological status.
The renal function was reduced in all the 15 cases and the liver function in 9. These functions gradually recovered along with gradual decrease of the dose. Two cases each manifested neurological and intestinal com-plications of Behcet disease during the treatment with cyclosporin A.
The findings indicate the systemic cyclosporin A is beneficial in reducing or preventing ocular attacks of Behcet disease on a long term basis. A careful observa-tion of patients is mandatory during the treatment because of possible side effects.
Rinsho Ganka (Jpn J Clin Ophthalmol) 40(5) : 465-468, 1986
Copyright © 1986, Igaku-Shoin Ltd. All rights reserved.