儿童系统性红斑狼疮32例临床分析

Clinical retrospective analysis of 32 systemic lupus erythematosus in Chinese children

目的探讨儿童系统性红斑狼疮的临床特点。方法对32例儿童系统性红斑狼疮进行临床分析。结果男女比例1∶4.3,平均年龄11.4岁。首发症状中非特异性症状及皮肤黏膜损害均为31%,关节肌肉症状为19%,血液系统损害及肾脏损害分别占13%、6%。后期血液及肾脏损害分别上升达72%、59%。8例肾活检结果中以Ⅳ型狼疮为主(88%)。32例病人中有9例在疾病早期曾有误诊,误诊率达28%。ANA、dsDNA阳性分别为87%、90%,C3降低为37%,狼疮细胞检测阳性率达58%,15例病人中14例(93%)EB病毒壳抗体IgG阳性。结论儿童系统性红斑狼疮男女发病差异较小;首发症状以非特异性症状及皮肤黏膜损害为主,后期则以血液及肾脏损害为主;其发病与EB病毒关系密切;该病临床表现多样,易误诊。

Objective To investigate the clinical characteristics of systemic lupus erythematosus （SLE） . Methods We retrospectively analyzed the clinical manifestations of 32 children diagnosed as SLE in our hospital between 1994 to 2003. Results Male to female ratio was 1： 4.3. The average age at presentation was 11.4 yrs. The common ini- tial manifestations of childhood SLE were non-specific （ 31% ）, cutaneous （ 31% ）, musculoskeletal and arthritic （19%）, hemotologic（ 13% ）, and renal（6% ）. Following the progression of the disease, the most cotumon manifestations were hemotologic （72%） and renal （59%）. Renal pathologic findings showed WHO Class 1V in 7 of 8 cases（88% ）. 9 of 32 patients were mis-diagnosed at early stage of the disease. Anti-nuclear antibodies（ ANA）, anti-double stranded DNA antibodies （dsDNA）, positive LE cell, and low C3 level were showed in 87%, 90%, 58% and 37 % of patients, respectively. EB-VCA antibodies were positive in 14 of 15 patients （93%）. Conclusion Compared with adult, there was less difference between male and female incidence in childhood-onset SLE. The most common initial manifestations were non-specific and cutaneous, while at later stage were hemotologic and renal. The possible relation between SLE and EB virus infection was suspected and further study needed to investigate. The manifestations were plethora and tuis-diagnosis at the early stage was not uncommon.