Evaluating Autoimmunity in Patients with Agammaglobulinemia

Document Type: Original Article


1 Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center, Tehran University of Medical Science, Tehran, Iran

2 Student Research Committee, Alborz University of Medical Sciences, Karaj, Iran


Background: Agammaglobulinemia is a primary immunodeficiency disorders (PID) which is identified by increased susceptibility to the bacterial infections, significant low antibodies and isohemagglutins and decreased peripheral B cells counts. Different clinical manifestations could be observed in these patients. Some of agammaglobulinemia patients manifest autoimmune disorders, while the association of autoimmunity and agammaglobulinemia is not clarified yet. In this study, we evaluated the frequency of autoimmunity in agammaglobulinemia patients and we compared their demographic, clinical and laboratory data in two groups of the patients with and without autoimmunity.
Material and Methods: The present study included 147 patients diagnosed with agammaglobulinemia who were diagnosed at the 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 assess the autoimmune complications.
Results: Among 147 agammaglobulinemia patients, we identified 21 (14.2%) (18 males and 3 females) agammaglobulinemia patients who had autoimmune disorders. In a total of the patients, the most prominent clinical presentation was respiratory infections (72.4%). Among autoimmune disorders, Juvenile Rheumatoid Arthritis was the most important autoimmunity (38%) following Immune thrombocytopenia 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 patients. Early diagnosis and proper management of autoimmunity in agammaglobulinemia cases lead to enhancement of patient's quality of life.