儿童系统性红斑狼疮12例序贯治疗疗效观察

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目的 应用系统性红斑狼疮活动指数 (SLEDAI)和系统性红斑狼疮国际临床合作组与美国风湿病学会损害指数 (DI)的方法指导儿童系统性红斑狼疮 (SLE)患儿序贯疗法。方法  12例儿童SLE采用序贯疗法。治疗前进行SLEDAI和DI评估。对SLEDAI和DI均存在肾脏损伤和 (或 )神经系统损伤的 5例 (Ⅰ组 )采用大剂量甲基泼尼松龙 (MP)冲击诱导 [15~ 30 (mg/kg) ],连续或隔日 ,共 3次 ,结束时口服泼尼松 1mg/ (kg·d) (平均40mg/d) 3~ 4周后 ,予以环磷酰胺 (CTX)静脉冲击治疗 [8~ 12mg/ (kg·d) ],连续 2d ,每 2周重复Ⅰ组 ,累计剂量≤ 15 0mg/kg ,以后每 3个月重复 1组 ,全部重复剂量≤ 5 0mg/kg ;同时给予羟基氯喹 (HCQ)≤ 5mg/ (kg·d)。CTX维持治疗结束 6~ 10个月后 ,口服雷公藤多甙 (Tw) 1~ 2mg/ (kg·d) 3~ 6个月。此间 ,泼尼松逐渐减量 ,以最小有效控制量长期维持。无肾脏损伤和 (或 )神经系统损伤的 7例 (Ⅱ组 )均采用口服泼尼松 1 5~ 2 0mg/ (kg·d) ,临床症状控制后 1个月逐渐减量 ,剂量减至 1 0mg/ (kg·d)时 ,加Tw(剂量同Ⅰ组 ,连续口服 4~ 9个月 ) ,同时给予HCQ(同Ⅰ组 ) ,当泼尼松减量至 0 5mg/ (kg·d)时 ,可重复应用Tw3~ 4个月。随访期间进行DI评估。随访结束时年龄 14~ 19岁 ,平均随访时间 Objective To guide the sequential therapy of children with systemic lupus erythematosus (SLE) by the method of systemic lupus erythematosus activity index (SLEDAI) and the international clinical cooperation group of systemic lupus erythematosus and American College of Rheumatology damage index (DI). Methods 12 cases of children with sequential therapy SLE. SLEDAI and DI assessments were performed prior to treatment. Five patients (Group I) with kidney damage and / or nervous system injury in both SLEDAI and DI were induced by high dose methylprednisolone (MP) shock [15-30 mg / kg] for consecutive or alternate days , 3 times at the end of the treatment. After oral administration of prednisone 1 mg / (kg · d) (mean 40 mg / d) at the end of 3 to 4 weeks, CTX venous pulse therapy [8 to 12 mg / (kg · d) ] For 2 consecutive days. Patients in group Ⅰ were repeated every 2 weeks, and the cumulative dose was ≤15 0 mg / kg. Patients in each group were repeated every 3 months for a total dose of ≤50 mg / kg. HCQ ≤5 mg / kg · D). CTX maintenance therapy for 6 to 10 months after the oral administration of tripterygium glycosides (Tw) 1 ~ 2mg / (kg · d) for 3 to 6 months. Here, prednisone tapering, the minimum effective control of the long-term maintenance. Seven patients without renal injury and / or nervous system injury (group Ⅱ) received oral prednisone 15 ~ 20 mg / (kg · d), and their dosage gradually decreased 1 month after the clinical symptoms were controlled (0 mg / (kg · d)), plus Tw (the same dose as the group Ⅰ, continuous oral administration of 4 to 9 months), given HCQ (with Ⅰ group), when the dose of prednisone reduced to 0 5mg / (kg · d) When, can be repeated application of Tw3 ~ 4 months. DI assessment during follow-up. At the end of follow-up, patients were 14 to 19 years of age with mean follow-up time
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