Recurrent parotid enlargement as an initial manifestation of Sjögren's syndrome in children: a case report

Abstract

Sjögren's syndrome (SS) is a chronic inflammatory autoimmune disease mainly affecting the salivary and lacrimal glands, usually occurring in adults. Sjögren's syndrome in children is a rare and often misdiagnosed condition. Recurrent parotid enlargement appears to be an important presenting sign. Previous studies in pediatric SS conclude that existent diagnostic criteria are of limited applicability in children, because of unreliable reporting of the patient's medical history and frequent absence of ocular symptoms. Diagnosis of Sjögren's syndrome can be confirmed in the presence of positive serologic testing, oral and ocular manifestations, and recurrent salivary gland enlargement. A 10-year-old African-American female presented to the Oral Medicine clinic with a chief complaint of episodic, sometimes painful, parotid swellings for the past 2 years. Prior to our oral medicine work-up, the patient was seen by pediatric medicine, ORL, rheumatology, and ophthalmology. Serology obtained 6 months prior to consultation was negative for autoimmune dysfunction. Laboratory analysis and clinical exam performed in recent visits revealed positive SS-A and SS-B antibodies and a negative Schirmer's score (greater than 5 mm). Computer tomographic images taken 2 months prior to presentation revealed bilateral parotid and submandibular gland enlargement with multiple cystic structures of varying size and parotid calcifications. Otherwise, the patient's past medical history was only significant for hospitalization at age 1 for Rous sarcoma virus. She denied taking any medications, and had no known drug allergies. Her family history was significant for systemic lupus erythematosus (father). Other than the parotid swellings, the patient's review of systems was negative, including no complaint of fevers, chills, malaise, anorexia, dysgeusia, xerostomia, or xerophthalmia. On physical exam the patient appeared well nourished, well developed, and in no apparent distress. Her vital signs were stable. Head and neck exam revealed bilateral nontender parotid area swellings measuring 4 cm in diameter on the right and 2 cm in diameter on the left. Gross exam of the cranial nerves revealed mild paresthesia of the right V-3 distribution. Intraorally, the patient exhibited dry mucosa and chronic caries. Review of a panoramic film showed cloudiness of the right mandibular ramus consistent with a soft-tissue swelling. Unstimulated and stimulated salivary flow tests were performed, resulting in no saliva production. Sjögren's syndrome has to be considered in the evaluation of bilateral parotid gland enlargement in children. Although a recent consensus was reached regarding the diagnosis of SS, specific criteria for children are absent. The late appearance of SS immune markers and the absence of ocular symptoms complicate the diagnosis.