High Response and Relapse Rate in Cyclosporine-Treated Minimal Change Disease and Focal Segmental Glomerulosclerosis in Taiwanese Patients

背景：環孢靈(CsA)曾應用於類固醇依賴型∕不反應型之微細病變(MCD)或局部性腎絲球硬化症(FSGS)病人。然而其應用於台灣病人之臨床經驗卻相當缺乏。方法：本研究收錄從2004年至2007年，回溯分析經腎切片證實為微細病變（l5人）及局部性腎絲球硬化症（7人）之病人接受環孢靈治療，其劑量為環孢靈3mg/Kg/day及類固醇10mg/day。仔細觀察其腎功能及尿蛋白變化，同時分析其臨床反應、預後及副作用。結果：二十二位患者中，類固醇依賴型佔了十七位(77.3%)，類固醇不反應型有五位(227%)。而微細病變族群中，環孢靈治療可引起緩解者有十一位(73%)，只有四位病人(27%)對環孢靈反應不佳。從環孢靈治療到疾病緩解之時間平均為5.0±1.8週。但有緩解之七位病人停用環孢靈，其中六位(85.7%)都復發，而復發時間發生在停藥20.0±4.1週內。環孢靈治療局部性腎絲球硬化症病人亦有類似之結果。相較於類固醇不反應型病人，對類固醇有反應者其對環孢靈之治療明顯有較佳之反應(P<0.05)。副作用包含了多毛症(50%)、牙齦腫大(27.2)、高血壓(9.1%)及高血鉀(4.5%)。在使用環孢靈治療期間並無病患發生腎功能異常。結論：低劑量環孢靈治療類固醇依賴型∕不反反應型之微細病變或及局部腎絲球硬化症病人是有效且安全的，但其停藥之後的復發率依然是相當高的。
Background: Cyclosporine (CsA) is used in steroid-resistant and dependent minimal change disease (MCD) and focal segmental glomerulosclerosis (FSGS). The clinical experience is limited in Taiwanese patients who received a low dose of CsA combined with corticosteroid. Materials and Methods: We conducted a retrospective case series study of biopsy-proven MCD (15 patients) and FSGS (7 patients) with CsA treatment between 2004 and 2007. CsA therapy was used in steroid-resistant (SR) or steroid-dependent (SD) patients. The treatment protocol consisted of CsA 3 mg/Kg/day and prednisolone 10 mg/day. The clinical features, outcome, and adverse effects were assessed in detail. Results: Twenty-two patients received CsA therapy during the study period. Seventeen patients (77.3%) were steroid dependent (SD) and five patients (22.7%) were steroid-resistant (SR). In the MCD group, CsA therapy induced remission in eleven patients (73%). The mean duration for CsA-responsiveness was 5.0±1.8 weeks. Out of seven patients who withdrew from CsA, six patients (85.7%) relapsed wit/tin 20.2±4.1 weeks. The experience in the FSGS group was similar. The mean time to ac/sieve CsA-responsiveness was 4.6±2.7 weeks. Four of four patients (100%) relapsed within 18.6±6.2 weeks. Previous "steroid responsiveness" was the most powerful predictor of a favorable CsA response (P<0.05). Hypertrichosis, gum hypertrophy, hypertension, and transient hyperkalemia occurred in 50%, 27.2%, 9.1%, and 4.5% of patients, respectively. No patient developed renal dysfunction in the observation period. Conclusion: A low dose of CsA combined with corticosteroid is effective and safe for treatment of SD/SR patients with MCD and FSGS in Taiwan. However the relapse rate remains high after withdrawal of CsA.