Clarifying the Diagnostic Pathway in Pediatric Sjögren's Syndrome in the Absence of Child-Specific Criteria: A Systematic Review

Background: Pediatric Sjögren's syndrome (pSS) diagnosis remains challenging due to its low prevalence, heterogeneous clinical presentation, and the lack of universally accepted pediatric-specific diagnostic criteria. This systematic review aims to clarify the diagnostic pathway in pSS, with emphasis on the utility of minor salivary gland biopsy. Methods: A systematic review was conducted in accordance with PRISMA guidelines. PubMed, Scopus, and Web of Science were searched up to November 2025. Observational, retrospective, cohort, cross-sectional studies, case series, and case reports were included. Risk of bias was assessed using the Joanna Briggs Institute Critical Appraisal Checklist. Results: Thirty-nine studies met the inclusion criteria. Recurrent or persistent parotitis emerged as the most frequent glandular manifestation. The most common extra-glandular features included joint involvement and lymphadenopathy. Serological analysis revealed positivity for anti-SSA in 64% and anti-SSB in 51% of cases, with antinuclear antibodies and rheumatoid factor detected in 69% and 53% of patients, respectively. Minor salivary gland biopsy yielded positive findings in approximately 70% of cases. Conclusions: Minor salivary gland biopsy appears to represent a key component of the diagnostic work-up, particularly in the absence of standardized pediatric criteria. When integrated with clinical and serological findings, it may facilitate earlier and more accurate diagnosis.