Original Article

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

Abstract

Background/objectives: CVID is known as the most prevalent symptomatic primary immunodeficiency (PID) characterized by heterozygous manifestations including several infectious and non-in-fectious complications. Accordingly, although the hallmark of this disease is the development of recurrent sinopulmonary infections, different types of autoimmunity are frequently reported in CVID subjects. So 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, the patients were then divided into two groups of autoimmunity and non-autoimmunity for further analyses.
Results: Among 301 CVID cases enrolled in this study, 81 (26.9%) had autoimmunity that was mostly manifested as autoimmune cytopenia; 21 (24%) out of these 81 individuals had immune thrombocytopenic purpura (ITP) and 14 (17.3%) patients showed autoimmune hemolytic anemia (AIHA). Moreover, Rheumatologic autoimmune disorders such as 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 in 6 subjects (7.4%) and vitiligo in 7 patients (8.6%) were the most observed gastrointestinal and dermatologic autoimmune disorders in this study. Accordingly, some of these conditions were concomitant in a single individual. Additionally, several 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 among CVID patients.

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IssueVol 3, No 2 (2020) QRcode
SectionOriginal Article
DOI https://doi.org/10.22034/igj.2020.227556.1038
Keywords
Autoimmune complications Common variable immunodeficiency Primary immunodeficiency immune thrombocytopenic purpura
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How to Cite
1.
Babaha F. Autoimmunity Complications in Common Variable Immunodeficiency Patients; an update from the Iranian registry. Immunol Genet J. 2020;3(2):29-38.