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

CHENG Sang; XUE Hai-Yan; CAO Lan-Fang.

doi:10.7499/j.issn.1008-8830.2402073

PDF(523 KB)

Chinese Journal of Contemporary Pediatrics ›› 2024, Vol. 26 ›› Issue (7) : 695-700. DOI: 10.7499/j.issn.1008-8830.2402073

CLINICAL RESEARCH

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

CHENG Sang, XUE Hai-Yan, CAO Lan-Fang.

Author information +

History +

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

Cite this article

EndNote

Ris (Procite)

Bibtex

Download Citations

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]. Chinese Journal of Contemporary Pediatrics. 2024, 26(7): 695-700 https://doi.org/10.7499/j.issn.1008-8830.2402073

References

1 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.
2 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.
3 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.
4 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.
5 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.
6 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.
7 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.
8 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.
9 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.
10 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.
11 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.
12 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.
13 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.
14 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.
15 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.
16 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.
17 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.
18 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.
19 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.
20 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.
21 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.
22 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.
23 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.
24 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.
25 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.
26 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.
27 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.