Autoimmune exocrinopathy presenting as recurrent parotitis of childhood.

OBJECTIVE: To describe a case of autoimmune exocrinopathy in a child at the age of 3 months who presented with the original diagnosis of recurrent parotitis.

DESIGN: This a case report of a 9-year-old girl with recurrent parotitis who was later found to have Sjögren's syndrome. The literature relating to primary Sjögren's syndrome in the pediatric population is reviewed.

SETTING: The patient was seen in the rheumatology and otolaryngology clinics at a university medical center.

INTERVENTIONS: This study did not address therapy.

MAIN OUTCOME MEASURE: Diagnosis of Sjögren's syndrome.

RESULTS: The patient had xerostomia, an abnormal result of a salivary gland biopsy, SSA and SSB antibodies, and the histocompatibility antigens HLA-B8 and HLA-DR3 that are associated with Sjögren's syndrome.

CONCLUSION: Primary Sjögren's syndrome should be considered in cases of recurrent parotitis of childhood. More studies are needed to assess the natural history of autoimmune exocrinopathy in children.