目的　了解小儿系统性红斑狼疮 (SL E)的特点。方法　对 85例 SL E儿的临床资料进行分析。结果　 85例中起病年龄 10 a以上者 6 6例 (77.6 % ) ,男女比为 1:6 .1,12例 (14.1% )家族中有结缔组织病史。最常见的表现是抗核抗体 (ANA )阳性 (91. 8% )、血沉增快(90 .6 % )、肾脏受累 (82 .3% )、发热 (82 .3% )、低补体血症(81.2 % )、蝶形红斑 (6 9.4% )、关节症状 (6 2 .3% )及血液系统损害 (6 2 .3% )。 18例 (2 1.2 % )起病时表现为单一系统损害。6例 (7.0 % )肾损害始终为唯一临床表现。经激素联合免疫抑制剂治疗 ,随访 0 .5～ 2 3a,临床缓解或病情波动者 49例 ,无 1例进入慢性肾功能衰竭 ,死亡 9例 ,失访 2 7例。结论　本病临床表现多样 ,肾损害发生率高 ,早期正规治疗疗效显著 Objective To understand the characteristics of pediatric systemic lupus erythematosus (SL E). Methods The clinical data of 85 children with SL E were analyzed. Results Among the 85 cases, there were 66 cases (77.6%) with a history of more than 10 years, the ratio of male to female was 1: 6. In 12 cases (14.1%), there was a history of connective tissue. The most common manifestations were ANA positive (91.8%), ESR (90.6%), kidney involvement (82.3%), fever (82.3%), hypocomplementemia (81.2%), butterfly erythema (6 9.4%), joint symptoms (62.3%) and hematological damage (62.3%). Eighteen patients (2 1.2%) presented with a single systemic lesion at onset. Six cases (7.0%) of kidney lesions remained the only clinical manifestation. Hormone combined immunosuppressive therapy, follow-up 0.5 ~ 2 3a, 49 cases of clinical remission or fluctuations in the disease, no one into chronic renal failure, 9 patients died, 27 cases were lost. Conclusion The clinical manifestations of this disease are diverse, the incidence of renal damage is high, and the effect of early formal treatment is significant