摘要
目的评估儿童系统性红斑狼疮(cSLE)患者在停用糖皮质激素(GC)后的长期预后。方法回顾性分析2006年1月至2020年12月在首都医科大学附属北京儿童医院确诊的15例cSLE患者的临床资料,均进行了长期随访,在18岁之前停用GC,并在停用GC后至少随访了1年。结果15例cSLE患儿中,诊断为狼疮性肾炎4例,神经精神狼疮3例。所有患者均接受口服泼尼松治疗,中位剂量为1.7(1.1,2.0)mg/(kg·d)。达到狼疮低疾病活动状态(LLDAS)的中位时间为30(18,36)个月,达到临床缓解的中位时间为38(26,46)个月。所有患者在停用GC前均保持临床缓解和血清学正常(抗dsDNA抗体阴性和补体水平正常)。GC治疗的持续时间为53(45,82)个月。在停用GC后,所有患者继续服用缓解病情的抗风湿药物(DMARDs)。停用GC后的中位随访时间为21(16,44)个月。1例患者在停用GC后第8个月出现轻度复发。结论cSLE患者在达到长期临床缓解后停用GC是可行的,但需要通过更大规模的临床研究进一步验证。
Objective To evaluate the long-term prognosis of childhood-onset systemic lupus erythematosus(cSLE)after discontinuation of glucocorticoids(GC)therapy.Methods The clinical data of 15 patients with cSLE who were diagnosed in Beijing Children′s Hospital,Capital Medical University from January 2006 to December 2020 were retrospectively analyzed.All the patients underwent long-term follow-up and their GC therapy was discontinued before the age of 18,and the patients were followed up for at least 1 year after discontinuation of GC therapy.Results Of the 15 patients with cSLE,4 cases were diagnosed with lupus nephritis and 3 with neuropsychiatric lupus.All the patients were treated with oral prednisone at a median dose of 1.7(1.1,2.0)mg/(kg·d).The median time to reach lupus low disease activity state(LLDAS)was 30(18,36)months,and the median time to reach clinical remission was 38(26,46)months.All the patients maintained clinical remission and serologically normal[anti-double-stranded deoxyribonucleic acid(dsDNA)antibodies-negative and normal complement levels]before discontinuation of GC therapy.The duration of GC therapy was 53(45,82)months.After GC was discontinued,all the patients continued to take disease-modifying anti-rheumatic drugs(DMARDs).The median follow-up time after discontinuing GC therapy was 21(16,44)months.One padiatric patient experienced a mild relapse at the 8th month after discontinuation of GC therapy.Conclusion It is feasible for the cSLE patients to discontinue GC therapy after achieving long-term clinical remission.However,larger scale clinical studies are needed to further validate the feasibility of discontinuing GC therapy in the cSLE patients.
作者
李士朋
邝伟英
邓江红
张俊梅
檀晓华
李超
李彩凤
LI Shipeng;KUANG Weiying;DENG Jianghong;ZHANG Junmei;TAN Xiaohua;LI Chao;LI Caifeng(Department of Rheumatology,Beijing Children′s Hospital,Capital Medical University,National Center for Children′s Health,Beijing 100045,China)
出处
《中国临床新医学》
2024年第9期972-976,共5页
CHINESE JOURNAL OF NEW CLINICAL MEDICINE
基金
北京市教育委员会科技计划重点项目(编号:KZ202210025037)。
关键词
儿童
系统性红斑狼疮
糖皮质激素
预后
Children
Systemic lupus erythematosus
Glucocorticoids(GS)
Prognosis
