Sjögren syndrome (SS) is an autoimmune disease of the exocrine glands resulting in the inflammatory infiltrate of the lacrimal and salivary glands. This glandular dysfunction leads to xerophthalmia (dry eyes) and keratoconjunctivitis sicca in the eyes and xerostomia (dry mouth). SS may also present with extra-glandular involvement including pulmonary (bronchiolitis, bronchiectasis, pneumonitis), renal (renal tubular acidosis), neurologic (neuropathy, cerebellar ataxia, encephalopathy), and cutaneous (vasculitis, purpura, xerosis) manifestations. SS may be primary or secondary to an autoimmune condition, most commonly systemic lupus erythematosus but also scleroderma and rheumatoid arthritis.

SS most commonly occurs in middle-aged adult women, and there is a similar predominance in pediatric SS patients, who typically present in late childhood / early preteen years with recurrent parotitis. Compared to adults, children with SS are less likely to experience keratoconjunctivitis sicca and xerostomia.

The American-European Consensus Group's (AECG) criteria for primary SS in adults have not been validated in children. AECG criteria include eye symptoms, oral symptoms, findings of keratoconjunctivitis sicca on Schirmer or Rose-Bengal testing, lymphocytic infiltrate of minor salivary glands on biopsy, positive antibodies to SSA or SSB, and salivary gland dysfunction. The addition of recurrent parotitis increases the criteria's sensitivity in children with SS. Given the decreased incidence of xerostomia in children with SS, minor salivary gland biopsy may not have a high yield.