PO:23:046 | Histopathological comparison between minor salivary gland biopsies from pediatric and adult patients with Sjögren’s disease: evidence of a more severe inflammatory profile in early-onset cases

Flavia Riccio¹, Mattia Neri², Martina Mellone¹, Immacolata Cozzolino³, Jacopo Ferro⁴, Valerio Gaetano Vellone⁴, Ciro Emiliano Boschetti⁵, Giuseppe Colella⁵, Nicola Laffi⁶, Pietro Buono⁷, Enrico Tirri⁸, Renato Franco³, Marco Gattorno², Francesco Ciccia¹, Clara Malattia², Saviana Gandolfo⁸ | ¹UOC di Reumatologia, Università degli Studi della Campania Luigi Vanvitelli, Napoli; ²UOC di Reumatologia e Malattie Autoinfiammatorie, Istituto Giannina Gaslini, Genova; ³UO di Anatomia Patologica, Università degli Studi della Campania Luigi Vanvitelli, Napoli; ⁴UOC di Anatomia Patologica, Istituto Giannina Gaslini Genova; ⁵UOC di Chirurgia Maxillo-facciale, Università degli Studi della Campania Luigi Vanvitelli, Napoli; ⁶UO di Odontoiatria, Istituto Giannina Gaslini, Genova; ⁷UOD Attività consultoriali e assistenza materno-infantile, Direttorato Generale per la Salute, Regione Campania, Napoli; ⁸UO di Reumatologia, Ospedale del Mare, Napoli, Italy

Background. Pediatric-onset Sjögren’s disease (pSjD) presents distinct clinical and serological features compared with the adult form (SjD). While adults more frequently exhibit sicca symptoms such as xerophthalmia and xerostomia, parotid swelling and cutaneous involvement are the most common clinical manifestations in pSjD. From a serological perspective, pediatric patients less frequently test positive for ANA and anti-SSA/SSB antibodies but tend to show higher levels of rheumatoid factor (RF). The aim of this study was to compare the histopathological characteristics of minor salivary gland (MSG) biopsies between pediatric and adult patients with SjD.

Materials and Methods. The study included 18 consecutive patients with pSjD followed at an Italian pediatric rheumatology center and 54 consecutively evaluated adult SjD patients from an adult rheumatology unit. All biopsy samples were examined by an expert pathologist at each site according to the EULAR recommendations for standardized histopathological assessment of minor salivary gland biopsies.

Results. The surface area of biopsy specimens did not differ significantly between the two groups (p = 0.551). Pediatric patients displayed significantly higher focus scores (p = 0.0227) and a greater prevalence of ectopic germinal centers, as evidenced by increased CD21 (52.94% vs. 16.22%, p = 0.005) and Bcl6 expression (47.06% vs. 8.11%, p = 0.0011). Lymphoepithelial lesions were also more frequent in the pediatric cohort (55.56% vs. 18.92%, p = 0.0065), as was fibrosis (66.67% vs. 29.73%, p = 0.031). No significant differences were observed for glandular atrophy (p = 0.687) or ductal dilation (p = 0.128). When stratified by disease onset relative to menarche, prepubertal-onset patients exhibited a higher frequency of germinal centers and lymphoepithelial lesions than those with postpubertal-onset disease (p < 0.05).

Conclusions. This study represents the first direct histopathological comparison of minor salivary gland biopsies between pediatric and adult patients with Sjögren’s disease. The findings confirm a more pronounced inflammatory pattern in pediatric patients, consistent with the higher clinical activity observed in this population. In particular, the increased frequency of ectopic germinal centers and lymphoepithelial lesions in prepubertal-onset cases suggests the possible involvement of age-dependent immunopathogenic mechanisms underlying early-onset pSjD. These results reinforce the pivotal role of minor salivary gland biopsy not only in diagnosis but also in disease prognostication, emphasizing the importance of early recognition and close clinical monitoring in pediatric patients.