Autoimmunity in Patients With Selective IgA Deficiency

Journal of Investigational Allergology and Clinical Immunology

Aghamohammadi A³  Rezaei N³  Mohammadi J²  Khazaei HA⁴  Torabi-Sagvand B³  Arandi N³  Mirminachi B³  Havaei A³  Masoom SN³  Shahinpour S³  Gharib B³  Abolhassani H¹ 

[1] Division of Clinical Immunology, Department of Laboratory Medicine, Karolinska Institute at Karolinska University Hospital Huddinge, Stockholm, Sweden;Department of Life Science, Faculty of New Science and Technology, University of Tehran, Tehran, Iran;Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran;Department of Immunology and Hematology, Zahedan Medical Sciences University, Zahedan, Iran

关键词: Regulatory T cells;    Switched memory B cell;    Immunologic characteristics;    Autoimmunity;    Selective IgA deficiency;   

Others  :  1183608

【 摘 要 】

Background and Objective: Selective immunoglobulin A deficiency (SIgAD) is the most common primary antibody deficiency. Patients with SIgAD have a greater risk of concomitant autoimmune disorders than healthy individuals. The exact mechanism underlying the relationship between autoimmunity and SIgAD is not fully understood. The aim of this study was to evaluate potential associations between autoimmunity and specific clinical or immunological findings in patients with SIgAD.

Methods: The study population comprised 57 symptomatic patients (65% males) with confirmed SIgAD who were referred to our center. Demographic data and history of autoimmunity were recorded both for patients and for their relatives. Comprehensive clinical and laboratory examinations were performed to investigate autoimmune complications in all the patients.

Results: Autoimmune disorders were documented in 17 cases (29.8%; 9 males and 8 females). The most common manifestations were thyroiditis, vitiligo, and hemolytic anemia (3 cases each). Ten patients (17.5%) had a family history of autoimmunity. Significant associations were detected between autoimmunity and increased duration of follow-up (P=.003), serum level of IgM (P=.01), regulatory T-cell count (P=.03), and class-switched memory B-cell count (P=.01). Four cases of autoimmune SIgAD (23.5%) progressed to common variable immunodeficiency during the follow-up period (P=.006).

Conclusions: Autoimmune disorders, autoimmune cytopenia, and Ig subclass deficiency can lead to severe clinical manifestations in patients with SIgAD. Therefore, immunologists and pediatricians should be aware of these conditions.