Evaluating Autoimmunity in Patients with Agammaglobulinemia

Introduction: Agammaglobulinemia is considered as a primary immunodeficiency disorder (PID) which is identified with increasing susceptibility to the bacterial infections, significant low antibodies and isohemagglutinins and decreasing peripheral B cells counts. It could be observed different clinical manifestations in these patients. Some of the patients with agammaglobulinemia manifest autoimmune disorders, while association of autoimmunity and agammaglobulinemia has not been clarified yet. In this study, we evaluated the frequency of autoimmunity in agammaglobulinemia patients and compared their demographic, clinical and laboratory data in two groups of the patients with and without autoimmunity. Methods: The present study included 147 patients with agammaglobulinemia who were diagnosed at Research Centre for Immunodeficiencies at the children’s medical Centre. A comprehensive history, demographic information, clinical manifestations, laboratory data were obtained from all patients to evaluate the autoimmune complications. Results: Among 147 agammaglobulinemia patients, we identified 21 patients (14.2%) who had autoimmune disorders (18 males and 3 females). Respiratory infections were the most prominent clinical symptom among all the patients (72.4%). Among autoimmune disorders, Juvenile Rheumatoid Arthritis (JRA) was the most important autoimmunity (38%) then, Immune Thrombocytopenia (ITP) with the frequency of 14%. Conclusion: Autoimmune diseases are not very common among agammaglobulinemia patients; however, these disorders should be considered as an important factor for management in the patients. Early diagnosis and suitable management of autoimmunity leads to enhancement of patient s life quality in agammaglobulinemia cases.