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Special Clinical Presentation in Patients with Selective IgA Deficiency: Autoimmunity Phenotype

Davoud Amirkashani1, Asghar Aghamohammadi1*, Hassan Abolhassani1*, Shervin Shahinpour1, Seyedeh Nina Masoom1 and Arash Havaei1
  • 1 Tehran University of Medical Science, Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children’s Medical Center, Iran

The most common primary antibody deficiency in Caucasians race is Selective immunoglobulin A deficiency(SIgAD). SIgAD Patients have been previously shown to have an increased risk for concomitant autoimmune disorders comparing to the normal population. The exact mechanism of relationship between autoimmunity and SIgAD is not fully understood yet. The aim of this study is to evaluate the probable associations between the autoimmunity and clinical or immunological findings in SIgAD patients. Fifty-seven symptomatic patients(65%males) with confirmed diagnosis of SIgAD who were referred to our center from 1984to2011 were enrolled in this study. Demographic data and history of autoimmunity in patients and their relatives were recorded and comprehensive clinical and laboratory examinations related to autoimmune complications were performed in all patients. Among 57 patients, autoimmune disorders were documented in 17 cases (29.8%, 9 males and 8females). Thyroiditis, vitiligo and autoimmune hemolytic anemia(AIHA) were the most common manifestations with 3 subjects for each disorder. Ten patients (17.5%) showed autoimmune disorders in their other family members. There were significant associations between increased serum level of IgM (P=0.01) and decreased WBC count (P=0.03) with the development of autoimmunity in our patients. Four autoimmune SIgAD cases (23.5%) evolved to common variable immunodeficiency during follow-up period (p=0.006). Regulatory T cells and switched memory B cells were reduced in the patients with autoimmunity significantly rather than remaining cases. Autoimmune disorders along with infections and allergic diseases appear to be among the most important clinical manifestations of SIgAD patients which should be consider by immunologist and pediatricians.

Keywords: Selective IgA deficiency, Autoimmunity, Immunologic characteristic, autoimmune thyroiditis, autoimmune hemolytic anemia

Conference: 15th International Congress of Immunology (ICI), Milan, Italy, 22 Aug - 27 Aug, 2013.

Presentation Type: Abstract

Topic: Adaptive Immunity

Citation: Amirkashani D, Aghamohammadi A, Abolhassani H, Shahinpour S, Masoom S and Havaei A (2013). Special Clinical Presentation in Patients with Selective IgA Deficiency: Autoimmunity Phenotype. Front. Immunol. Conference Abstract: 15th International Congress of Immunology (ICI). doi: 10.3389/conf.fimmu.2013.02.00303

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Received: 16 Mar 2013; Published Online: 22 Aug 2013.

* Correspondence:
Prof. Asghar Aghamohammadi, Tehran University of Medical Science, Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children’s Medical Center, Tehran, Iran, aghamohammadi@tums.ac.ir
Dr. Hassan Abolhassani, Tehran University of Medical Science, Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children’s Medical Center, Tehran, Iran, hassan.abolhassani@ki.se