儿童原发性干燥综合征并发肾脏损害的特点分析  

Analysis of the characteristics of juvenile primary Sj(?)gren's syndrome with renal damage

作　　者：茹晋丽[1] 曾小峰[1] 张立民[2] 何金昌[3] 朱佳[1] 

机构地区：[1]中国医学科学院中国协和医科大学北京协和医院风湿免疫科 [2]山东省泰安市中心医院风湿科 [3]四川省攀枝化市中心医院风湿科

出　　处：《中华风湿病学杂志》2007年第5期295-298,共4页Chinese Journal of Rheumatology

摘　　要：目的探讨出现肾脏损害的儿童原发性干燥综合征(pSS)患者的临床表现、病理特点、治疗及预后。方法回顾性分析北京协和医院门诊和住院诊治的儿童pSS 24例,并发肾损害者10例,收集相关资料,并与无肾损害者进行比较。结果在10例肾损害患儿组,9例为Ⅰ型肾小管酸中毒(RTA),5例有低钾性麻痹,3例伴有肾结石,1例肾性尿崩,3例有蛋白尿。在3例行肾脏穿刺的患儿中,肾脏病理均有肾间质病变,2例同时有肾小球受累,其中1例与系统性红斑狼疮重叠。肾损害组与无肾损害组的两组患者的临床表现如:口干症状、龋齿情况、浅表淋巴结肿大、关节症状、皮疹以及实验室指标红细胞沉降率、血球蛋白、自身抗体检测之间比较,差异无统计学意义。联合激素、免疫抑制剂及对症治疗均可使其临床症状减轻、红细胞沉降率、血球蛋白下降。结论肾脏损害是儿童原发性干燥综合征常见的系统损害之一,其中以肾小管酸中毒最常见;肾小球也可受累,但少见。有系统受累者,治疗多倾向于激素联合免疫抑制剂,短期可取得显著疗效。Objective To study the renal damage of patients with juvenile primary Sjogren＇s syndrome （pSS） and its clinical manifestations, pathologic characteristics with biopsy, treatment and prognosis. Methods Ten patients with juvenile pSS complicated with renal impairments were retrospectively analyzed. Data of these 10 patients were compared with those without renal impairments. Results Ten patients complicated with renal impairments in 24 patients with juvenile pSS, 9 of them presented with type Ⅰ renal tubular acidosis （RTA）, 5 with hypokalemia paralysis,3 with calcification of the renal tissue, 3 with positive urine protein, 1 with diabetes insipidus. There was no significant difference between patients with renal impairments and those without. Three patients underwent kidney biopsy that showed chronic interstitial nephritis （CIN） with extensive lymphoplasmic cell infiltration. Two patients had glomerular lesions and one of them was diagnosed as pSS overlaped with systemic lupus erythematosus （SLE）. Steroid and immunosuppressive agents had significantly alleviated symptoms and the hypergammaglobulinemia was significantly improved. Conclusion Renal impairment may be the major complication in juvenile pSS. The major clinical manifestations are RTA and the glomeruli are involved occasionally. Treatment with steroid and immunosuppressive agents should be given to those who have evidence of systemic involvement.

关 键 词：干燥综合征 儿童 回顾性研究 肾脏损害 

分 类 号：R725.9[医药卫生—儿科] R726.9[医药卫生—临床医学]