Autoimmune Exocrinopathy Presenting as Recurrent Parotitis of Childhood

To describe a case of autoimmune exocrinopathy in a child at the age of 3 months who presented with the origianl diagnosis of recurrent parotitis. —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. —The patient was seen in the rheumatology and otolaryngology clinics at a university medical center. —This study did not address therapy. —Diagnosis of Sjögren's syndrome. —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. —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.