甲泼尼龙和环磷酰胺冲击治疗儿童重症系统性红斑狼疮的临床研究  

Methylprednisolone and cyclophosphamide pulse therapy of severe systemic lupus erythematosus in children

作　　者：胡坚[1] 李崇巍[1] 张瑄[1] 皮士红[1] 赵津生[1] 陈晓颖[1] 刘嫣[1] 

机构地区：[1]天津市儿童医院内科风湿免疫组,300074

出　　处：《中华儿科杂志》2003年第6期430-434,共5页Chinese Journal of Pediatrics

摘　　要：目的　了解甲泼尼龙 (MP)和环磷酰胺 (CTX)冲击治疗儿童重症系统性红斑狼疮(SeverJOSLE)的临床效果和转归。方法　确诊的儿童系统性红斑狼疮 30例 ,初治 2 2例 ,其中狼疮性肾炎 (LN) 2 0例 ,9例纳入前病程 3～ 1 2个月。 30例患儿中 ,2 3例有肾损伤的临床表现 ,其中 ,WHO Ⅳ型 4例 ,WHO Ⅱ型 2例 ,WHO Ⅴ型 1例 ,WHO Ⅲ型 1例 ,7例肾损伤同时伴 1项或以上的神经系统损伤的临床表现 :舞蹈症、癫样发作、脑血管意外 (CAV)和器质性脑综合征 (OBS)等 ;有神经系统症状不伴肾损伤临床表现的 9例 ;3例狼疮危象 (lupuscrisis)。其余 7例不伴肾损伤临床表现 ,仅有轻微或一过性中枢神经系统损伤。根据治疗方法分为 3组 :A组 ,1 8例 ,临床或病理证实合并LN或精神神经性狼疮 (NPLE) ,采用MP +CTX冲击治疗 ;B组 ,7例 ,临床无LN和 (或 )NPLE ,MP冲击诱导后 ,小剂量泼尼松维持治疗 ,并以雷公藤多甙 (Twhf)接续治疗 ;C组 ,5例 ,临床以LN为主 ,糖皮质激素 (GS)治疗。观察各组的近期、远期疗效和远期临床转归。结果　平均随访时间为 (37 2± 2 4 8)个月。近期疗效 :A组与B组间SLEDAI 2K的差别主要是肾脏权重计分 ,9个月时 ,A组的变化最大 ,计分与B组相似 ,C组SLEDAI 2K计分在中度活动范围 ,主要是肾脏的权重结果。远期?Objective To study the effect of methylprednisolone (MP) and cyclophosphamide (CPA) intermittent intravenous pulse therapy and the clinical prognosis in children with severe juvenile onset systemic lupus erythematosus (JOSLE) Methods Thirty patients with JOSLE, diagnosed by clinical, laboratory or renal histological examinations, were enrolled in this study Of the 30 patients, 27 were females and 3 were males, the mean age was (12±3) years, and 20 of the 22 patients who had undergone initial therapy had LN, and the clinical courses before being involved in the study were 3 to 12 months in nine patients Twenty three of the 30 patients had clinical manifestations of renal damages, of whom 4 patients were proven by initial renal biopsy to have WHO type Ⅳ, 2 had type Ⅱ,1 had type Ⅴ and 1 had type Ⅲ, and 7 patients had one or more manifestations of central nervous system, including chorea, seizures, cerebrovascular accident(CVA)and organic brain syndrome (OBS), simultaneously, 9 patients had nervous system symptoms without the clinical manifestations of renal damages, 3 patients had lupus crisis, 7 patients did not have any manifestations of renal or neurological damages According to the protocol of the therapy, the patients were divided into 3 groups: group A( n = 18)patients were treated with MP plus CPA intermittent intravenous pulse for children with lupus nephritis, and with or without neuropsychiatric lupus erythematosus (NPLE), group B ( n = 7) with pulsed doses of MP, followed by prednisone and tripterygium wilfordii hook f(T whf )for patients without renal or central nerves system damage, and group C ( n =5) with prednisone alone for patients with LN determined by clinical and laboratory features The effects of those regimes and the clinical prognosis were observed Results On short term follow up, the SLEDAI 2K (by weight of the renal damage) showed significant difference between group A and group B, but there was no significant difference at the 9th months of the ther

关 键 词：甲泼尼龙 环磷酰胺 冲击治疗 儿童 系统性红斑狼疮 临床研究 

分 类 号：R725.9[医药卫生—儿科]