Minor salivary gland biopsy in the diagnosis of definite ocular sarcoidosis in paediatric granulomatous uveitis.

ABSTRACT:

Background

Non-infectious paediatric granulomatous uveitis (PGU) is a rare disease that is idiopathic in more than half of affected children. The diagnosis of definite ocular sarcoidosis (OS) must be supported by the presence of non-caseating granulomas detected in biopsy, and is therefore a challenge in children with PGU. This study investigated the utility of minor salivary gland biopsy (MSGB) in the diagnosis of definite OS in PGU.

Methods

Twenty-six consecutive children with PGU diagnosed between 2018 and 2023 and with a systematically performed MSGB within 3 months of the diagnosis were enrolled.

Results

The median age at PGU diagnosis was 11.6 (4.2-16.5) years, and 54% of the children were boys. PGU consisted mainly of bilateral (92%) pan-uveitis (96%). MSGB detected non-caseating granulomas (MSGB+) in 12/26 (46%) children. In all, 13 of the 26 (50%) children were diagnosed with definite OS, and 8 (31%) had idiopathic uveitis. MSGB had a sensitivity of 92%, and a NPV of 93% in the diagnosis of definite OS in children with PGU. Compared to MSGB- children, those who were MSGB + were more frequently older than 10 years of age at diagnosis (p = 0.02), had a higher rate of general signs (p = 0.003), extra-ocular organ involvement (p = 0.005) and polyclonal hypergammaglobulinaemia (p = 0.03). The most frequent extra-ocular organ involvements at OS diagnosis were renal (46%) and thoracic (46%). First-line therapy was systemic corticosteroids in 88% of the children. During a median follow-up time of 3.1 (0.6-6.3) years after PGU diagnosis, 88% of the children needed methotrexate and/or anti-tumour necrosis factor-alpha therapy to achieve inactive uveitis.

Conclusions

MSGB is useful to improve the diagnosis of OS and to reduce the incidence of uveitis considered idiopathic in PGU. MSGB could be considered in PGU patients, particularly those > 10 years of age with general signs and/or hypergammaglobulinaemia.

SUBMITTER: Etchandy L 

PROVIDER: S-EPMC11892283 | biostudies-literature | 2025 Mar

REPOSITORIES: biostudies-literature