More severe parotid gland histopathology in paediatric-onset than in adult-onset Sjögren's disease

Geertje Elizabeth Legger; Uzma Nakshbandi; Martha S van Ginkel; Silvia C Liefers; Lisette de Wolff; Alja J Stel; Wineke Armbrust; Fred K L Spijkervet; Arjan Vissink; Suzanne Arends; Hendrika Bootsma; Bert van der Vegt; Gwenny M Verstappen; Frans G M Kroese

doi:10.1136/rmdopen-2024-004201

More severe parotid gland histopathology in paediatric-onset than in adult-onset Sjögren's disease

RMD Open. 2024 Aug 13;10(3):e004201. doi: 10.1136/rmdopen-2024-004201.

Affiliations

¹ Pediatric Rheumatology, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands g.e.legger@umcg.nl.
² Rheumatology and Clinical Immunology, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands.
³ Oral and Maxillofacial Surgery, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands.
⁴ Pediatric Rheumatology, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands.
⁵ Pathology and Medical Biology, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands.

Abstract

Objectives: The aim of this study was to assess the histopathological features of the parotid glands in patients with paediatric-onset Sjögren's disease (pedSjD) in comparison to patients with adult-onset Sjögren's disease (adSjD).

Methods: This study was performed in Groningen, the Netherlands. Patients with pedSjD from a diagnostic paediatric cohort (n=19), patients with adSjD from a diagnostic adult cohort (n=32) and patients with adSjD who participated in a clinical trial (n=42) with a baseline parotid gland biopsy were included. Parotid gland biopsies were analysed after (immuno)histological staining for SjD-related histopathological markers and compared between groups.

Results: All characteristic histopathological features of adSjD were also observed in pedSjD. There were no significant differences in lymphoepithelial lesions or immunoglobulin A (IgA)/IgG plasma cell shift between the pedSjD and the adSjD cohorts. However, compared with the diagnostic adSjD cohort (with comparable total EULAR Sjögren's Syndrome Disease Activity Index (ESSDAI) scores), pedSjD showed more severe lymphocytic infiltration as reflected by a higher focus score (p=0.003), a higher relative surface area of CD45+ infiltrate (p=0.041), higher numbers of B and T lymphocytes/mm² (p=0.004 and p=0.029, respectively), a higher B/T lymphocyte ratio (p=0.013), higher numbers of CD21+ follicular dendritic cell networks/mm² (p=0.029) and germinal centres (GC)/mm² (p=0.002). Compared with the trial adSjD cohort, with significant higher total ESSDAI scores (p=0.001), only the B/T lymphocyte ratio and numbers of GC/mm² were significantly higher in the pedSjD cohort (p=0.023 and p=0.018, respectively).

Conclusion: Patients with pedSjD exhibit more pronounced histopathological features compared with patients with adSjD at diagnosis. Notably, the histopathology of patients with pedSjD aligns more closely with that observed in an adSjD clinical trial cohort, with even stronger B lymphocyte involvement.

Keywords: Autoimmune Diseases; B-Lymphocytes; Child; Inflammation; Sjogren's Syndrome.

MeSH terms

Adolescent
Adult
Age of Onset*
Aged
Biomarkers
Biopsy
Child
Female
Humans
Male
Middle Aged
Netherlands / epidemiology
Parotid Gland* / pathology
Severity of Illness Index
Sjogren's Syndrome* / diagnosis
Sjogren's Syndrome* / pathology
Young Adult

Substances

Biomarkers