探讨以组织细胞坏死性淋巴结炎(HNL)为首发临床表现的儿童系统性红斑狼疮(cSLE)患儿的临床特征及其预后。

选择2013年1月至2023年1月宁夏医科大学总医院收治的3例以HNL为首发临床表现的cSLE患儿(患儿1～3)为研究对象。采用回顾性分析方法，对其临床病例资料进行分析。分别以"组织细胞坏死性淋巴结炎""系统性红斑狼疮""儿童""histiocytic necrotizing lymphadenitis""systemic lupus erythematosus""children"为中文和英文关键词，在中国知网、万方数据库、PubMed数据库中检索相关文献，并进行文献复习。检索时间设定为2013年3月至2023年3月。本研究经宁夏医科大学总医院伦理委员会批准(伦理编号：KYLL-2023-0011)，监护人对患儿的诊治均知情同意。

①患儿1为男性，患儿2、3为女性，确诊时病程为11 d至1个月，均以发热起病伴淋巴结大(直径>2 cm)为主诉；白细胞计数(WBC)均降低，以淋巴细胞占比为主；淋巴结活组织病理学检查结果为HNL。患儿1～3的抗核抗体(ANA)均呈高滴度、双联DNA(ds-DNA)抗体(＋)。患儿1尿常规结果显示，WBC升高、血尿、面部可见蝶形红斑、补体3(C3)水平下降，系统性红斑狼疮(SLE)病情活动性评分为16分。患儿2合并中枢神经病变、血尿、关节炎，SLE病情活动性评分为19分。患儿3合并血尿、面部可见蝶形红斑、C3水平下降，SLE病情活动性评分为12分。②文献复习结果：根据本研究设定的文献检索策略，检索涉及关于以HNL为首发临床表现的cSLE患儿临床研究的文献为3篇，均为中文文献，涉及患儿为3例(患儿4～6)。患儿4被诊断为HNL时，同时被诊断为SLE，患儿5、6均为被诊断为HNL 6个月后，逐渐演变为SLE。

淋巴结受累可能是SLE多个受累器官之一，其中以HNL为首发临床表现的cSLE患儿的脏器损伤较轻，预后较好。

To investigate clinical features and prognosis of childhood systemic lupus erythematosus (cSLE) with histiocytic necrotizing lymphadenitis (HNL) as the first clinical manifestation.

From January 2013 to January 2023, 3 cases of cSLE with HNL as the first clinical manifestation (children 1-3) were selected in the study. The clinical data of these 3 cases were analyzed by retrospective analysis. Then, with " histiocytic necrotizing lymphadenitis" " systemic lupus erythematosus" " children" as Chinese and English keywords, the relevant pieces of literature were searched in CNKI, Wanfang Database and PubMed, and every piece of literature was reviewed. This study was approved by the Ethics Committee of the General Hospital of Ningxia Medical University (Approval No. KYLL-2023-0011), and the guardians gave informed consent to the diagnosis and treatment of children.

Among these 3 children, Child 1 was boy and child 2, were girl. The course of the disease was 11 d-1 month at the time of diagnosis. The chief complaint was fever with lymphadenopathy (diameter>2 cm). The white blood cell count (WBC) decreased, with lymphocytes accounting for the majority. Pathological examination results of lymph node biopsy were HNL. All three children showed high titer of antinuclear antibody (ANA) and double-stranded DNA (ds-DNA) was positive. Child 1 showed elevated WBC, hematuria, butterfly erythema on the face and decreased level of complement 3 (C3), and systemic lupus erythematosus (SLE) activity score was 16. Child 2 combined central neuropathy, hematuria and arthritis, and SLE activity score was 19. Child 3 showed hematuria, butterfly erythema on the face, decreased level of C3, and SLE activity score was 12. ②According to the literature retrieval strategy set in this study, the literature review results were as follows. three pieces of relevant Chinese literature were retrieved, including all of children were about the cSLE disease with HNL as the first manifestation. One case (child 4) was diagnosed with HNL and SLE simultaneously, while the other two cases (child 5-6) gradually developed into SLE six months after being diagnosed with HNL.

Lymph nodes may be one of the multiple organs arrested involved in SLE, and children with cSLE who present with HNL as the first manifestation may have relatively mild organ damage and a better prognosis.