Sjögren Syndrome in an Adolescent with IgA Deficiency

Abstract

A 16-year-old girl presented with bilateral parotitis, and further investigation revealed that the patient had serologic evidence of an undifferentiated connective tissue disease. Biopsy revealed a lymphocytic infiltrate of salivary glands, and a positive Schirmer's test indicated decreased tear production. A diagnosis of Sjögren syndrome was made. Further evaluation revealed that the patient had selective IgA deficiency. Patients with selective IgA deficiency are known to be at an increased risk for autoimmune diseases. While Sjögren syndrome is rare in 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.