Autoimmunity Complications in Common Variable Immunodeficiency Patients; an update from the Iranian registry

Document Type: Original Article

Author

Department of Immunology, faculty of medical sciences, Tarbiat Modares University

10.22034/igj.2020.227556.1038

Abstract

Introduction: Common Variable Immunodeficiency (CVID) is the most prevalent symptomatic primary immunodeficiency (PID) characterized by heterozygous manifestations, including infectious and non-infectious complications. The hallmark of disease is the development of recurrent sinopulmonary infections; however, different types of autoimmunity are frequently reported in CVID subjects. In this study, we aimed to provide an update report on various autoimmunity manifestations in a group of CVID patients in Iran.
Methods: Demographic, clinical and immunologic data of Iranian CVID cases who were followed up at children’s medical center were collected. Based on the presence of autoimmunity, patients were divided into two groups of autoimmunity and non-autoimmunity for further analyses.
Results: Among 301 CVID cases enrolled, 81 (26.9%) had autoimmunity which was mostly manifested as autoimmune cytopenia; 21 (24%) out of 81 individuals had immune thrombocytopenic purpura (ITP) and 14 (17.3%) patients showed autoimmune hemolytic anemia (AIHA). Rheumatologic autoimmune disorders like Juvenile Idiopathic Arthritis (JIA) and Juvenile Rheumatoid Arthritis (JRA) were observed in 7 (8.6%) and 5 (6.2%) individuals, respectively. Also, Inflammatory bowel disease 6 (7.4%) and vitiligo 7 (8.6%) were the most observed gastrointestinal and dermatologic autoimmune disorders in this study. Some of these conditions were concomitant in a single individual. Additionally, significant correlations were observed between autoimmunity and other complications including sinusitis (P=0.04), Bronchiectasis (P=0.002), Chronic diarrhea (P=0.000) and thrombocytopenia (P=0.000).
Conclusion: Autoimmunity and its association with other clinical manifestations should be paid more attention in CVID patients.

Keywords


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