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ArticleArthritis research & therapy2026

Minor salivary gland biopsies in childhood-onset Sjögren's disease show greater inflammation and more frequent fibrotic changes than in adult-onset Sjögren's disease.

Flavia Riccio, Silvana Ancona, Vincenzo Luca Lentini, Jacopo Ferro, Valerio Gaetano Vellone, Nicola Laffi, Enrico Tirri, Marco Gattorno, Aroldo Rizzo, Francesco Ciccia and 2 more

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Article in Arthritis research & therapy, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.

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12 authors.

Flavia RiccioDepartment of Precision Medicine, Rheumatology Section, University of Campania Luigi Vanvitelli, Via Pansini, 5, Naples, 80131, Italy.
Silvana AnconaUOC Reumatologia e Malattie Autoinfiammatorie, IRCCS Istituto Giannina Gaslini, Genoa, Italy.
Vincenzo Luca LentiniPathology Unit, Ospedali Riuniti Villa-Sofia - Cervello, Palermo, Italy.
Jacopo FerroPathology Unit, IRCCS Istituto Giannina Gaslini, Genoa, Italy.
Valerio Gaetano VellonePathology Unit, IRCCS Istituto Giannina Gaslini, Genoa, Italy.
Nicola LaffiPediatric Dentistry and Orthodontics Unit, IRCCS Istituto Giannina Gaslini, Genoa, Italy.
Enrico TirriRheumatology Unit, Ospedale del Mare, Naples, Italy.
Marco GattornoUOC Reumatologia e Malattie Autoinfiammatorie, IRCCS Istituto Giannina Gaslini, Genoa, Italy.
Aroldo RizzoPathology Unit, Ospedali Riuniti Villa-Sofia - Cervello, Palermo, Italy.
Francesco CicciaDepartment of Precision Medicine, Rheumatology Section, University of Campania Luigi Vanvitelli, Via Pansini, 5, Naples, 80131, Italy. francesco.ciccia@unicampania.it.
Clara Malattia *UOC Reumatologia e Malattie Autoinfiammatorie, IRCCS Istituto Giannina Gaslini, Genoa, Italy.
Saviana Gandolfo *Department of Precision Medicine, Rheumatology Section, University of Campania Luigi Vanvitelli, Via Pansini, 5, Naples, 80131, Italy.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

objectivesTo provide the first histopathological characterization of minor salivary gland (MSG) biopsies in childhood-onset Sjögren's disease (cSjD) in comparison with adult Sjögren's disease (SjD).

methodsMSG biopsies obtained by eighteen consecutive children fulfilling the 2016 ACR/EULAR classification criteria for SjD and 55 consecutive adult SjD patients were analysed. Clinical features, ESSDAI, ESSPRI, and salivary gland histopathology were collected according to EULAR standards, including focus score (FS), lymphoepithelial lesions (LELs), and ectopic germinal centers (GCs) by immunohistochemistry.

resultsCompared with adults, children showed a more inflammatory phenotype. Recurrent parotid swelling was significantly more frequent in cSjD (38.9% vs. 5.5%, p = 0.0015), whereas xerostomia and xerophthalmia predominated in adults (p < 0.0001 for both). Palpable purpura was more common in children (44.4% vs. 1.8%, p < 0.0001), while fatigue and myalgia were rare (p = 0.0058 and p < 0.0001, respectively). Rheumatoid factor was more prevalent in cSjD (77.7% vs. 30.9%, p = 0.0072), whereas anti-SSA antibodies were less frequent (p = 0.0029). ESSDAI scores were higher (p = 0.0001) and ESSPRI lower (p < 0.0001) in children, reflecting greater systemic inflammation but lower subjective symptom burden. Pediatric biopsies exhibited higher FS than adults (median 3.4 vs. 1.0, p = 0.0002), together with more frequent GCs (44.4% vs. 5.5%, p = 0.00034), LELs (61.1% vs. 5.5%, p < 0.0001), and fibrotic changes (66.7% vs. 27.3%, p = 0.0048). Quantitative immunohistochemistry further showed significantly increased CD3 and CD20 lymphocytic infiltrates in cSjD biopsies, together with an increased CD20/(CD20 + CD3) proportion (all p < 0.0001).

conclusionsMSG biopsies in cSjD show a more inflammatory, lymphoid-organizing B-enriched phenotype, with more frequent fibrosis than in adults. These findings are in keeping with previous observations in pediatric parotid tissue and highlight frequent fibrotic remodelling alongside marked inflammation despite shorter disease duration in children.

Indexed as

InflammationSalivary Glands, MinorSjogren's SyndromeAdolescentAdultAgedAge of OnsetBiopsyChildChild, PreschoolFemaleFibrosisHumansImmunohistochemistryMaleMiddle AgedBiopsyChildhood-onsetFibrosisGerminal centersHistopathologyMinor salivary glandSjögren’s disease

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.