小儿系统性红斑狼疮的特点

Clinical features of systemic lupus erythematosus in children

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 。

Objective To investigate the clinical characteristics of systemic lupus erythematosus(SLE) in children. Methods The clinical data of 85 children with SLE were stu died prospectively. Results Of 85 cases,66 patients(77%) were beyond 10 years old at the onset of the illness.The ratio of male to female was 1 to 6.1 .Twelve of the 85 patients(14.1%)had relatives with a connective tissue disease.The frequent manifestations included the presence of antinuclear antibody(91.8%),the quickened erythrocyte sedimentation rate(90.6%),renal involvement(82.3%),fever(82.3%),hypocomplementemia(81.2%),malar rash(69.4%),joint symptoms(62.3%)and hematologic disorder(62.3%).At the onset of disea se,18 patients(21.2%)only had the symptom of isolated systemic damage and 6 patients(7%) had a renal damage as their only manifestation during the whole period of the disease.During clinical follow up for 0.5 to 23 years with steroid and immunosuppressants treatment,49 patients were in complete or partial remission,9 patients died and 27 lost follow up. Conclusion The clinical picture in patient with SLE consists of a variety of symptoms with a high incidence of renal impairment.If treated early and regularly,they can get a favorable therapeutic response.