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Autoimmunity in Primary Antibody Deficiencies Publisher Pubmed



Azizi G1, 5 ; Ahmadi M6 ; Abolhassani H5, 9, 10 ; Yazdani R2, 3 ; Mohammadi H4 ; Mirshafiey A5, 6 ; Rezaei N5, 7, 11 ; Aghamohammadi A5, 8
Authors
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Authors Affiliations
  1. 1. Department of Laboratory Medicine, Imam Hassan Mojtaba Hospital, Alborz University of Medical Sciences, Karaj, Iran
  2. 2. Department of Immunology, School of Medicine, Isfahan University of Medical Sciences, Iran
  3. 3. Molecular Immunology Interest Group (MIIG), Universal Scientific Education and Research Network (USERN), Isfahan, Iran
  4. 4. Department of Immunology, School of Medicine, Tabriz University of Medical Sciences, Tabriz, Iran
  5. 5. Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center Hospital, 62 Qarib St., Keshavarz Blvd., Tehran, 14194, Iran
  6. 6. Department of Immunology, School of Public Health, Iran
  7. 7. Department of Immunology, School of Medicine, Tehran University of Medical Sciences, Iran
  8. 8. Primary Immunodeficiency Diseases Network (PIDNet), Universal Scientific Education and Research Network (USERN), Tehran, Iran
  9. 9. Division of Clinical Immunology, Department of Laboratory Medicine, Karolinska Institute, Karolinska University Hospital Huddinge, Sweden
  10. 10. Primary Immunodeficiency Diseases Network (PIDNet), Universal Scientific Education and Research Network (USERN), Stockholm, Sweden
  11. 11. Network of Immunity in Infection, Malignancy, and Autoimmunity (NIIMA), Universal Scientific Education and Research Network (USERN), Boston, MA, United States

Source: International Archives of Allergy and Immunology Published:2017


Abstract

Primary antibody deficiencies (PADs) are the most common inherited primary immunodeficiencies in humans, characterized by hypogammaglobulinemia, an inability to produce specific antibodies, and recurrent infections mainly caused by encapsulated bacteria. However, it has been shown that inflammatory disorders, granulomatous lesions, lymphoproliferative diseases, cancer, and autoimmunity are associated with the various types of PAD. Both systemic and organ-specific autoimmune diseases could be attributed to B-cell defects in PAD patients. Immune thrombocytopenic purpura and autoimmune hemolytic anemia are the most common autoimmune disorders in this group of patients. The aim of this review is to describe the proposed mechanisms for autoimmunity and to review the literature with respect to the reported autoimmune disorders in each type of PAD. © 2017 S. Karger AG, Basel.
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