儿童系统性红斑狼疮合并干燥综合征的临床特点及唇腺病理特征分析

Clinical characteristics and labial gland pathological features in children with systemic lupus erythematosus complicated by Sjögren's syndrome

目的分析儿童系统性红斑狼疮(systemic lupus erythematosus,SLE)合并干燥综合征(Sjögren's syndrome,SS)的临床表现、实验室特点及唇腺病理特征。方法回顾性选取2013年1月—2022年12月上海交通大学医学院附属仁济医院收治的102例行唇腺活检的SLE患儿为研究对象,根据是否合并SS,将SLE患儿分为SLE合并SS组(SLE-SS)(60例)和单纯SLE组(42例)。根据唇腺病理灶性指数(focus score,FS),将SLE-SS患儿分为FS≥4亚组(26例)和FS<4亚组(34例)。比较分析各组患儿的临床资料。结果与单纯SLE组相比,SLE-SS组患儿皮肤黏膜受累少,更易出现抗SSA抗体、抗SSB抗体阳性和类风湿因子升高(P<0.05),两组治疗方案比较差异无统计学意义(P>0.05)。与FS<4亚组相比,FS≥4亚组患儿关节肌肉受累更常见(P<0.05),两亚组在SLE疾病活动指数及其他重要脏器受累方面比较差异无统计学意义(P>0.05)。结论合并SS的SLE患儿较少累及皮肤黏膜,具有特异性的血清学特征;FS≥4的SLE-SS患儿更易出现关节肌肉受累,但FS高低与病情活动及其他重要脏器损伤无关。

Objective To study the clinical manifestations,laboratory features,and labial gland pathological features in children with systemic lupus erythematosus(SLE)complicated by Sjögren's syndrome(SS).Methods A retrospective analysis was conducted on 102 children with SLE who underwent labial gland biopsies at Renji Hospital,Shanghai Jiao Tong University School of Medicine from January 2013 to December 2022.The children were divided into two groups based on the presence of SS:the SLE with SS group(SLE-SS;60 children)and the SLE-only group(42 children).According to the focus score(FS)of the labial glands,children in the SLE-SS group were further subdivided into FS≥4 subgroup(26 children)and FS<4 subgroup(34 children).The clinical data of the groups were compared.Results Compared to the SLE-only group,children in the SLE-SS group had less skin and mucosal involvement,were more likely to have positive anti-SSA and anti-SSB antibodies,and had higher levels of rheumatoid factor(P<0.05).There was no significant difference in treatment protocols between the two groups(P>0.05).Compared to the FS<4 subgroup,the FS≥4 subgroup had more frequent musculoskeletal involvement(P<0.05),but there was no significant difference in SLE disease activity or other major organ involvement between the subgroups(P>0.05).Conclusions Children with SLE complicated by SS are less likely to have skin and mucous membrane involvement and exhibit specific serological characteristics.The SLE-SS children with an FS≥4 are more likely to experience musculoskeletal involvement.However,FS is not associated with disease activity or other significant organ damage.