SJOGREN SYNDROME IN AN ADOLESCENT WITH IGA DEFICIENCY

A 16-year-old girl presented with bilateral parotitis, and further inv estigation revealed that the patient had serologic evidence of an undi fferentiated connective tissue disease. Biopsy revealed a lymphocytic infiltrate of salivary glands, and a positive Schirmer's test indicate d decreased tear production. A diagnosis of Sjorgren syndrome was made . Further evaluation revealed that the patient had selective IgA defic iency. Patients with selective IgA deficiency are known to be at an in creased risk for autoimmune diseases. While Sjogren syndrome is rare i n childhood and adolescence, the presence of selective IgA deficiency is thought to produce clinical conditions that may favor the access of infectious agents and macromolecules to the host. These substances in turn may activate the immune system, ultimately leading to autoimmune disease.