Pusongchai Tasama, Jungthirapanich Jaakchai, Khositseth Sookkasem
Department of Pediatrics, Faculty of Medicine, Thammasat University, Patumthani, Thailand.
J Med Assoc Thai. 2010 Dec;93 Suppl 7:S283-93.
Systemic lupus erythematosus (SLE) is a common autoimmune disease in children. Current standard therapies carry high adverse effects. Refractory SLE to conventional therapies is not uncommon. Rituximab, anti-CD20 monoclonal antibody, has been used as an adjunctive therapy in children with refractory SLE with limited reports. This study described pediatric SLE patients in a single center, Thailand. To determine the clinical manifestations, treatments, and outcome of SLE patients, the authors retrospectively studied 19 patients (age <15 years) diagnosed with SLE at Thammasat University hospital, from January 01, 2002 through March 31, 2010. The mean age was 12.9 +/- 1.6 years; mean follow-up 3.3 +/- 2.6 years. Seventeen (89.5%) patients were female. Clinical manifestations were hematological (89.5%), dermatologic (73.7%), and renal involvement (68.4%). SLE was diagnosed 1 year after systemic onset juvenile rheumatoid arthiris in one patient. Lupus nephritis (LN) class II was observed in 30.8%, class III (15.4%), and class IV (53.8%) of patients with LN. Overall, mean SLEDAI score at presentation was 14.9 +/- 2.2 and significantly decreased to 6.8 +/- 1.6 (p < 0.0001) at 1 month after treatment. Complete remission at 1 year demonstrated in 11 (68.7%) patients. Infection was the most common complication followed by ophthalmological complications. All patients survived during follow-up period. Rituximab induced remission of SLE after refractory diffuse alveolar hemorrhage in one patient, and rapidly progressive glomerulonephirits leading to end stage renal failure in one patient. Clinical outcome of pediatric SLE was favorable in the present study. Complications from corticosteroid and anti-inflammatory therapy were high. Rituximab may be a good adjunctive therapy for refractory SLE in children. Large controlled trials to establish safety profile and optimal regimen of rituximab in childhood SLE are required.
系统性红斑狼疮（SLE）是儿童常见的自身免疫性疾病。目前的标准疗法不良反应严重。常规疗法治疗无效的SLE并不少见。利妥昔单抗，一种抗CD20单克隆抗体，已被用作难治性SLE患儿的辅助治疗，但相关报道有限。本研究描述了泰国一个单中心的儿童SLE患者情况。为确定SLE患者的临床表现、治疗方法及预后，作者回顾性研究了2002年1月1日至2010年3月31日在泰国法政大学医院诊断为SLE的19例（年龄<15岁）患者。平均年龄为12.9±1.6岁；平均随访3.3±2.6年。17例（89.5%）为女性。临床表现包括血液系统（89.5%）、皮肤（73.7%）及肾脏受累（68.4%）。1例患者在系统性起病的幼年类风湿关节炎1年后被诊断为SLE。狼疮性肾炎（LN）Ⅱ级在LN患者中占30.8%，Ⅲ级（15.4%），Ⅳ级（53.8%）。总体而言，就诊时SLEDAI平均评分为14.9±2.2，治疗1个月后显著降至6.8±1.6（p<0.0001）。11例（68.7%）患者在1年时达到完全缓解。感染是最常见的并发症，其次是眼部并发症。所有患者在随访期间均存活。利妥昔单抗使1例难治性弥漫性肺泡出血患者的SLE病情缓解，另1例快速进展性肾小球肾炎导致终末期肾衰竭的患者病情缓解。本研究中儿童SLE的临床预后良好。皮质类固醇和抗炎治疗的并发症发生率较高。利妥昔单抗可能是儿童难治性SLE的一种良好辅助治疗方法。需要进行大型对照试验以确定利妥昔单抗在儿童SLE中的安全性及最佳治疗方案。
