儿童系统性红斑狼疮33例临床分析

Clinical analysis of 33 cases of pediatric systemic lupus erythematosus

关键词:  儿童  系统性红斑狼疮  临床特征

Key words:  children  systemic lupus erythematosus  clinical features

摘要:

[目的]探讨儿童系统性红斑狼疮（SLE）的临床特征、治疗及预后。[方法]回顾性分析33例SLE患儿的临床资料。33例患儿首发症状以面部红斑、发热及关节炎为主；临床表现中肾脏受累87.88%，血液系统受累81.82%。病情活动性(SLEDAI积分)以中至重度活动为主（84.85％）。实验室检查以补体C3降低（93.94%），ANA阳性（90.91%）和ds-DNA阳性（78.79%）为主。14例肾脏病理活检中，Ⅳ型占42.86%。[结果]糖皮质激素联合免疫抑制剂治疗后，病情缓解；17例重症病例，在甲泼尼龙冲击治疗后快速控制病情。5年生存率85.71%（12/14）。[结论]儿童SLE起病形式多样，临床表现复杂，早期诊断困难；儿童SLE最易累及肾脏，早期肾脏活检，联合免疫抑制剂正规治疗，可改善预后；甲泼尼龙冲击治疗可快速控制病情。

Abstract:

[Objective]To summarize the clinical features, therapy and prognosis of systemic lupus erythematosus (SLE) in children. [Methods]The clinical data of 33 cases of systemic lupus erythematosus in children were reviewed retrospectively. Skin eruption was the most common initial manifestation followed by fever and arthritic. The commonly involved organs included kidney (87.88%) and hematologic system (81.82%). Activities in disease (SLEDAI SCORE) in the most cases were at moderate to severe levels (account for 84.85％). Among the laboratory parameters tested decreased serum levels of C3 was shown to have the highest positive rate of 93.94%, followed by antinuclear antibody (ANA) (90.91%) and anti-double stranded DNA (ds-DNA) (78.79%). Activities in disease (SLEDAI score) in the most cases were at mild to moderate levels. The most common renal pathology in lupus nephritis children is WHO class Ⅳ (42.86%). [Results]The conditions were improved in patients treated combined with immunosuppressive agents. Seventeen severe cases of SLE were treated with the methyl prednisone pulse therapy. The clinical symptoms, signs and laboratory tests were improved obviously within1-3 courses of the treatment. The 5-year survival of SLE children was 85.71%. [Conclusions]The manifestations of pediatric SLE are various and multisystem involvement is common. The initial manifestations of pediatric SLE are various, early stage diagnosis is difficult. It is important for the patients with1upus nephritis to have renal biopsy early, and treated combined with various immunosuppressive agents regularly in order to improve prognosis and survival rate. Patients with SLE having multisystem involvement are critical cases and the methyl prednisone shock therapy could control disease rapidly.