A Case of Sjogren's Syndrome Associated with Common Variable Immunodeficiency / 대한내과학회지

ABSTRACT

Common variable immunodeficiency (CVID) is a heterogeneous group of primary immune deficiencies in adults characterized by hypogammaglobulinemia, recurrent bacterial infections, and a higher incidence of autoimmune diseases. More than 25% of CVID patients also have autoimmune diseases such as autoimmune hemolytic anemia, immune thrombocytopenic purpura, rheumatoid arthritis, and systemic lupus erythematosus. However, the pathogenesis of autoimmunity in CVID remains obscure. We report a 56-year-old woman with CVID and Sjogren's syndrome. In addition to a long history of recurrent upper respiratory infections, acute gastroenteritis, and cellulitis, she has also suffered from persistent xerostomia and xerophthalmia for the past 10 years. Serologic studies revealed hypogammaglobulinemia (low levels of IgG, IgA, and IgM in serum) and the presence of anti-Ro antibodies, and salivary scintigraphy indicated salivary gland involvement. These findings led to a diagnosis of CVID and Sjogren's syndrome, which was treated by monthly intravenous immunoglobulin therapy.