Primary Sjogrens Syndrome in a Child

ogrens Syndrome (SS) is an uncommon condition in the pediatric age group. SS is a chronic autoimmune disorder mainly affects salivary and lacrimal glands, with varying degrees of systemic involvement. SS occurs usually after 40 years and nine times more common in females (1, 2). Primary SS, is not associated with any other illness, and secondary develops in the presence of autoimmune disorder such as rheumatoid arthritis, lupus, scleroderma or polymyositis (1). Clinically the hallmark symptoms of SS are dry mouth (xerostomia) and dry eyes (xerophthalmia). Recurrent parotid swelling is the common feature in children unlike in adults (1, 3). We report a nine -year-old patient with primary SS who presented with dry mouth, dry eyes, and recurrent parotid swelling. A nine-year-old boy presented with recurrent swelling of bilateral parotid glands since one year, dryness of mouth and eyes since six months ago. There was no history of joint pains, jaundice, drug ingestion, skin lesions, radiation or family history of autoimmune disorder. His weight and height were in 25th percentile. On examination he had dry oral mucosa, dry fissured tongue (Figure 1) and bilateral parotid enlargement. His conjunctiva was lusterless, tear film non uniform and break-up time was less than ten seconds. The rest of the systemic examination was normal. Investigations: Hb 12 gms/dL, WBC 8800 cells/cu.mm with normal differentiation, platelet count 390.000/cu mm, ESR 35 mm in first hour, urea 21 units/L, creatinine 0.7 mg/dL, sodium 139 mEq/L, potassium 3.9 mEq/L, AST 26 units/L, ALT 18 units/L and Amylase 42 units/L. His serology for RF, HCV, Hepatitis B, HIV, CRP, ANA, anti-double stranded DNA, Anti-SSA, Anti-SSB were negative. Ultrasonography of the parotid, submandibular and lacrimal glands showed hypoechoic areas with enlargement. MRI showed enlarged and hyper intense parotid, submandibular, sublingual and lacrimal glands on T1 images (Figure 2). His Schirmers test was positive (Right eye 3 mm, left eye 4 mm at 5 minutes), flourescin staining demonstrated superficial punctate keratitis. Minor salivary gland biopsy from lip revealed salivary acini and periductal lymphocytic and plasma cell infiltration (Figure 1). Our child fulfilled 4 AEC criteria for SS out of 6 with histopathological evidence. Child was started on refresh tear eye drops and advised to come for regular follow up.