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

doi:10.7499/j.issn.1008-8830.2402073

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摘要目的分析儿童系统性红斑狼疮（systemic lupus erythematosus, SLE）合并干燥综合征（Sj?gren'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。

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	程桑
	薛海燕
	曹兰芳

关键词 ：干燥综合征, 系统性红斑狼疮, 临床特征, 灶性指数, 儿童

Abstract：Objective To study the clinical manifestations, laboratory features, and labial gland pathological features in children with systemic lupus erythematosus (SLE) complicated by Sj?gren'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.

Key words： Sj?gren's syndrome Systemic lupus erythematosus Clinical characteristics Focus score Child

收稿日期: 2024-02-27

通讯作者: 曹兰芳，女，主任医师。Email：clf530417@126.com。 E-mail: clf530417@126.com

作者简介: 程桑，女，硕士，主治医师；

引用本文:

程桑,薛海燕,曹兰芳. 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[J]. 中国当代儿科杂志, 2024, 26(7): 695-700.

CHENG Sang,XUE Hai-Yan,CAO Lan-Fang.. Clinical characteristics and labial gland pathological features in children with systemic lupus erythematosus complicated by Sj?gren's syndrome[J]. CJCP, 2024, 26(7): 695-700.

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	Mariette X, Criswell LA. Primary Sj?gren's syndrome[J]. N Engl J Med, 2018, 378(10): 931-939. PMID: 29514034. DOI: 10.1056/NEJMcp1702514.
	Yao Q, Altman RD, Wang X. Systemic lupus erythematosus with Sj?gren syndrome compared to systemic lupus erythematosus alone: a meta-analysis[J]. J Clin Rheumatol, 2012, 18(1): 28-32. PMID: 22157271. DOI: 10.1097/RHU.0b013e31823ecbdf.
	Bautista-Vargas M, Vivas AJ, Tobón GJ. Minor salivary gland biopsy: its role in the classification and prognosis of Sj?gren's syndrome[J]. Autoimmun Rev, 2020, 19(12): 102690. PMID: 33099041. DOI: 10.1016/j.autrev.2020.102690.
	Ramos-Casals M, Acar-Denizli N, Vissink A, et al. Childhood-onset of primary Sj?gren's syndrome: phenotypic characterization at diagnosis of 158 children[J]. Rheumatology (Oxford), 2021, 60(10): 4558-4567. PMID: 33493333. DOI: 10.1093/rheumatology/keab032.
	Hochberg MC. Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus[J]. Arthritis Rheum, 1997, 40(9): 1725. PMID: 9324032. DOI: 10.1002/art.1780400928.
	Petri M, Orbai AM, Alarcón GS, et al. Derivation and validation of the Systemic Lupus International Collaborating Clinics classification criteria for systemic lupus erythematosus[J]. Arthritis Rheum, 2012, 64(8): 2677-2686. PMID: 22553077. PMCID: PMC3409311. DOI: 10.1002/art.34473.
	Aringer M, Costenbader K, Daikh D, et al. 2019 European League Against Rheumatism/American College of Rheumatology classification criteria for systemic lupus erythematosus[J]. Ann Rheum Dis, 2019, 78(9): 1151-1159. PMID: 31383717. DOI: 10.1136/annrheumdis-2018-214819.
	Shiboski CH, Shiboski SC, Seror R, et al. 2016 American College of Rheumatology/European League Against Rheumatism classification criteria for primary Sj?gren's syndrome: a consensus and data-driven methodology involving three international patient cohorts[J]. Arthritis Rheumatol, 2017, 69(1): 35-45. PMID: 27785888. PMCID: PMC5650478. DOI: 10.1002/art.39859.
	Tomiita M, Kobayashi I, Itoh Y, et al. Clinical practice guidance for Sj?gren's syndrome in pediatric patients (2018): summarized and updated[J]. Mod Rheumatol, 2021, 31(2): 283-293. PMID: 32856968. DOI: 10.1080/14397595.2020.1816319.
	Chisholm DM, Waterhouse JP, Mason DK. Lymphocytic sialadenitis in the major and minor glands: a correlation in postmortem subjects[J]. J Clin Pathol, 1970, 23(8): 690-694. PMID: 5488040. PMCID: PMC476869. DOI: 10.1136/jcp.23.8.690.
	Pasoto SG, Adriano de Oliveira Martins V, Bonfa E. Sj?gren's syndrome and systemic lupus erythematosus: links and risks[J]. Open Access Rheumatol, 2019, 11: 33-45. PMID: 30774485. PMCID: PMC6357904. DOI: 10.2147/OARRR.S167783.
	Sieiro Santos C, Moriano Morales C, álvarez Castro C, et al. Polyautoimmunity in systemic lupus erythematosus: secondary Sjogren syndrome[J]. Z Rheumatol, 2023, 82(Suppl 1): 68-73. PMID: 34287668. DOI: 10.1007/s00393-021-01051-x.
	Legger GE, Erdtsieck MB, de Wolff L, et al. Differences in presentation between paediatric- and adult-onset primary Sj?gren's syndrome patients[J]. Clin Exp Rheumatol, 2021, 39 Suppl 133(6): 85-92. PMID: 34796855. DOI: 10.55563/clinexprheumatol/vxe6h0.
	Hou L, Wang N, Zhao C, et al. A single-center study of clinical features of pediatric Sj?gren's syndrome[J]. Pediatr Rheumatol Online J, 2023, 21(1): 119. PMID: 37833819. PMCID: PMC10571342. DOI: 10.1186/s12969-023-00902-y.
	Xu D, Tian X, Zhang W, et al. Sjogren's syndrome-onset lupus patients have distinctive clinical manifestations and benign prognosis: a case-control study[J]. Lupus, 2010, 19(2): 197-200. PMID: 19900977. DOI: 10.1177/0961203309348235.
	Ruacho G, Kvarnstr?m M, Zickert A, et al. Sj?gren syndrome in systemic lupus erythematosus: a subset characterized by a systemic inflammatory state[J]. J Rheumatol, 2020, 47(6): 865-875. PMID: 31523050. DOI: 10.3899/jrheum.190250.
	Ponticelli C, Doria A, Moroni G. Renal disorders in rheumatologic diseases: the spectrum is changing (part 1: connective tissue diseases)[J]. J Nephrol, 2021, 34(4): 1069-1080. PMID: 32529559. DOI: 10.1007/s40620-020-00772-7.
	Aiyegbusi O, McGregor L, McGeoch L, et al. Renal disease in primary Sj?gren's syndrome[J]. Rheumatol Ther, 2021, 8(1): 63-80. PMID: 33367966. PMCID: PMC7991017. DOI: 10.1007/s40744-020-00264-x.
	Thatayatikom A, Jun I, Bhattacharyya I, et al. The diagnostic performance of early Sj?gren's syndrome autoantibodies in juvenile Sj?gren's syndrome: the University of Florida pediatric cohort study[J]. Front Immunol, 2021, 12: 704193. PMID: 34249010. PMCID: PMC8267463. DOI: 10.3389/fimmu.2021.704193.
	Vílchez-Oya F, Balastegui Martin H, García-Martínez E, et al. Not all autoantibodies are clinically relevant. Classic and novel autoantibodies in Sj?gren's syndrome: a critical review[J]. Front Immunol, 2022, 13: 1003054. PMID: 36325321. PMCID: PMC9619091. DOI: 10.3389/fimmu.2022.1003054.
	Liu C, Jin Y, Huang H, et al. Clinical and laboratory features of childhood-onset primary Sj?gren's syndrome: a retrospective study from China[J]. Front Pediatr, 2023, 10: 1044812. PMID: 36683822. PMCID: PMC9846242. DOI: 10.3389/fped.2022.1044812.
	Sharma R, Chaudhari KS, Kurien BT, et al. Sj?gren syndrome without focal lymphocytic infiltration of the salivary glands[J]. J Rheumatol, 2020, 47(3): 394-399. PMID: 31092717. PMCID: PMC7304293. DOI: 10.3899/jrheum.181443.
	Carvajal Alegria G, Depinoy T, Devauchelle-Pensec V, et al. Diagnostic utility of a second minor salivary gland biopsy in patients with suspected Sj?gren's syndrome: a retrospective cohort study[J]. Joint Bone Spine, 2023, 90(1): 105459. PMID: 36108904. DOI: 10.1016/j.jbspin.2022.105459.
	Edelstein R, Kilipiris GE, Machalekova K, et al. Accuracy of minor salivary gland biopsy in the diagnosis of Sj?gren syndrome[J]. Bratisl Lek Listy, 2021, 122(7): 454-460. PMID: 34161112. DOI: 10.4149/BLL_2021_075.
	Kakugawa T, Sakamoto N, Ishimoto H, et al. Lymphocytic focus score is positively related to airway and interstitial lung diseases in primary Sj?gren's syndrome[J]. Respir Med, 2018, 137: 95-102. PMID: 29605220. DOI: 10.1016/j.rmed.2018.02.023.
	Chatzis L, Goules AV, Pezoulas V, et al. A biomarker for lymphoma development in Sjogren's syndrome: salivary gland focus score[J]. J Autoimmun, 2021, 121: 102648. PMID: 34029875. DOI: 10.1016/j.jaut.2021.102648.
	Kroese FGM, Haacke EA, Bombardieri M. The role of salivary gland histopathology in primary Sj?gren's syndrome: promises and pitfalls[J]. Clin Exp Rheumatol, 2018, 36 Suppl 112(3): 222-233. PMID: 30156550.