Sicca syndrome and salivary gland infiltration in children with autoimmune disorders: when can we diagnose Sjögren syndrome?

ABSTRACT

OBJECTIVES: To analyse the initial presentation and outcome of children with a diagnosis of childhood-onset Sjögren's syndrome (SS) in a paediatric referral care center. To study whether the diagnosis was made in accordance with the most recent criteria of paediatric SS and to compare our patients to those reported in the literature. METHODS: We retrospectively analysed the clinical, histological and laboratory features of patients seen over a period of 15 years and diagnosed with SS before the age of 16. RESULTS: Eight patients had a diagnosis of SS in childhood and were followed for up to 14 years. Diagnosis of SS was based on histological evidence of salivary gland involvement in all patients with or without presence of specific autoantibodies (anti-SSA and -SSB). Sicca syndrome as a presenting symptom occurred in only 2/8 of children, recurrent parotid swelling in 3/8; whereas anti-SSA/SSB antibodies and typical salivary-gland histology were found in 6/8 patients. Five children fulfilled the proposed paediatric criteria for SS. Three patients did not fulfill the paediatric criteria but disclosed typical histology findings. Two patients developed overlapping lupus nephritis or autoimmune hepatitis years following diagnosis of SS. CONCLUSIONS: Childhood-onset SS is an heterogeneous disease in its presentation and outcome. The diagnosis may be discussed in some patients who do not fulfill the proposed diagnosis criteria, even though they disclose sicca syndrome and typical immunologic and histological findings. Some patients with typical SS may develop overlapping lupus disease over time.