Tehran University of Medical Sciences

Science Communicator Platform

Stay connected! Follow us on X network (Twitter):
Share this content! On (X network) By
Autoimmunity in a Cohort of 471 Patients With Primary Antibody Deficiencies Publisher Pubmed



Azizi G1, 2, 3, 4 ; Tavakol M1, 5 ; Rafiemanesh H6, 7 ; Kiaee F3 ; Yazdani R3 ; Heydari A3 ; Abouhamzeh K3 ; Anvari P3 ; Mohammadikhajehdehi S3 ; Sharifia L3 ; Bagheri Y8, 9 ; Mohammadi H10 ; Abolhassani H3, 4, 11 ; Aghamohammadi A3, 4
Authors
Show Affiliations
Authors Affiliations
  1. 1. Non-Communicable Diseases Research Center, Alborz University of Medical Sciences, Karaj, Iran
  2. 2. Department of Laboratory Medicine, Imam Hassan Mojtaba Hospital, Alborz University of Medical Sciences, Karaj, Iran
  3. 3. Research Center for Immunodeficiencies, Children’s Medical Center, Tehran University of Medical Sciences, Tehran, Iran
  4. 4. Primary Immunodeficiency Diseases Network (PIDNet), Universal Scientific Education and Research Network (USERN), Tehran, Iran
  5. 5. Department of Allergy and Clinical Immunology, Shahid Bahonar Hospital, Alborz University of Medical Sciences, Karaj, Iran
  6. 6. Students’ Research Committee, School of Public Health, Shahid Beheshti University of Medical Sciences, Tehran, Iran
  7. 7. Department of Epidemiology, School of Public Health, Shahid Beheshti University of Medical Sciences, Tehran, Iran
  8. 8. Student Research Committee, Golstan University of Medical Sciences, Gorgan, Iran
  9. 9. Department of Allergy and Clinical Immunology, Iran University of Medical Sciences, Tehran, Iran
  10. 10. Department of Immunology, School of Medicine, Tabriz University of Medical Sciences, Tabriz, Iran
  11. 11. Division of Clinical Immunology, Department of Laboratory Medicine, Karolinska Institute at Karolinska University Hospital Huddinge, Stockholm, Sweden

Source: Expert Review of Clinical Immunology Published:2017


Abstract

Objectives: The aim of this study was to evaluate the frequency of autoimmunity in primary antibody deficiency (PAD). Methods: A total of 471 patients with PADs enrolled in this retrospective cohort study. For all patients’ demographic information, clinical records and laboratory data were collected to investigate autoimmune complications. Results: Autoimmune disorders as the first presentation of immunodeficiency were recorded in 11 patients (2.5%). History of autoimmunity was recorded in 125 patients during the course of the disease (26.5%). The frequency of autoimmunity in common variable immune deficiency (32.0%) was higher than other forms of PADs. The most common autoimmune manifestations were reported to be autoimmune gastrointestinal disease and autoimmune cytopenias. Among patients with autoimmunity, 87 patients (69.6%) had a history of one autoimmune disorder, while 38 patients (30.4%) had a history of multiple autoimmunities. The immune thrombocytopenic purpura and autoimmune hemolytic anemia were the most two concomitant autoimmune disorders in 16 (42.1%) of 38 patients with multiple autoimmunities. Comparing the frequency of Tregs in PAD patients with autoimmunity showed that, patients with multiple autoimmunities had lower Tregs than those with single autoimmunity (p = 0.017). Conclusion: It is important that non-immunologist physicians be alert of the associated autoimmunity with PADs in order to reduce the diagnostic delay and establish timely immunoglobulin replacement therapy in these patients. © 2017 Informa UK Limited, trading as Taylor & Francis Group.
Other Related Docs
12. Infectious and Noninfectious Pulmonary Complications in Patients With Primary Immunodeficiency Disorders, Journal of Investigational Allergology and Clinical Immunology (2017)
13. Role of Rare Immune Cells in Common Variable Immunodeficiency, Pediatric Allergy and Immunology (2022)
16. Application of Flow Cytometry in Predominantly Antibody Deficiencies, Endocrine# Metabolic and Immune Disorders - Drug Targets (2021)
21. The Use of Immunoglobulin Therapy in Primary Immunodeficiency Diseases, Endocrine# Metabolic and Immune Disorders - Drug Targets (2016)
24. Comparison of Common Monogenic Defects in a Large Predominantly Antibody Deficiency Cohort, Journal of Allergy and Clinical Immunology: In Practice (2019)
25. T-Cell Abnormalities in Common Variable Immunodeficiency, Journal of Investigational Allergology and Clinical Immunology (2016)
28. A Review on Defects of Dendritic Cells in Common Variable Immunodeficiency, Endocrine# Metabolic and Immune Disorders - Drug Targets (2017)
32. Primary Antibody Deficiency in a Tertiary Referral Hospital: A 30-Year Experiment, Journal of Investigational Allergology and Clinical Immunology (2015)
34. Respiratory Complications in Patients With Hyper Igm Syndrome, Journal of Clinical Immunology (2019)
39. The Heterogeneous Pathogenesis of Selective Immunoglobulin a Deficiency, International Archives of Allergy and Immunology (2019)
40. Spectrum of Phenotypes Associated With Mutations in Lrba, Journal of Clinical Immunology (2016)
42. Autoimmunity in Patients With Selective Iga Deficiency, Journal of Investigational Allergology and Clinical Immunology (2015)
43. Autoimmunity in Primary T-Cell Immunodeficiencies, Expert Review of Clinical Immunology (2016)
49. Agammaglobulinemia: Epidemiology, Pathogenesis, Clinical Phenotype, Diagnosis, Prognosis and Management, Endocrine# Metabolic and Immune Disorders - Drug Targets (2020)