Fatema Sadaat Rizvi1, Hamed Zainaldain1, Hosein Rafiemanesh2, Mahnaz Jamee3,4, Nikoo Hossein-Khannazer5, Haleh Hamedifar6,7, Araz Sabzevari6,8, Reza Yazdani1, Hassan Abolhassani9, Asghar Aghamohammadi1, Gholamreza Azizi4. 1. Research Center for Immunodeficiencies, Children's Medical Center, Tehran University of Medical Sciences , Tehran, Iran. 2. Student Research Committee, Department of Epidemiology, School of Public Health and Safety, Shahid Beheshti University of Medical Sciences , Tehran, Iran. 3. Student Research Committee, Alborz University of Medical Sciences , Karaj, Iran. 4. Non-communicable Diseases Research Center, Alborz University of Medical Sciences , Karaj, Iran. 5. Gastroenterology and Liver Diseases Research Center, Research Institute for Gastroenterology and Liver Diseases, Shahid Beheshti University of Medical Sciences , Tehran, Iran. 6. CinnaGen Medical Biotechnology Research Center, Alborz University of Medical Sciences , Karaj, Iran. 7. CinnaGen Research and Production Co ., Alborz, Iran. 8. Orchid Pharmed Company , Tehran, Iran. 9. Division of Clinical Immunology, Department of Laboratory Medicine, Karolinska Institute at Karolinska University Hospital Huddinge , Stockholm, Sweden.
Abstract
Objectives: Common variable immunodeficiency (CVID) is the most common symptomatic inborn error of immunity characterized by variable clinical manifestations. Methods: Web of Science, Scopus, and PubMed databases were searched systemically to find eligible studies from the earliest available date to February 2020 with standard keywords. Pooled estimates of the autoimmunity prevalence and the corresponding 95% confidence intervals (CI) were calculated using random-effects models. Results: The overall prevalence of autoimmunity was 29.8% (95% CI: 26.4-33.3; I2 = 82.8%). The prevalences of hematologic autoimmune diseases, autoimmune gastrointestinal disorders, autoimmune rheumatologic disorders, autoimmune skin disorders, and autoimmune endocrinopathy in CVID patients were 18.9%, 11.5%, 6.4%, 5.9%), and 2.5%, respectively. There were significantly higher lymphocyte, CD3 + T cell, and CD4 + T cell count among CVID patients without autoimmunity (p< 0.05). Furthermore, failure to thrive, organomegaly, enteropathy, and meningitis was significantly higher in CVID patients with autoimmunity(p< 0.05). Conclusions: Many CVID patients could present with autoimmunity as part of the disease or even as the first or only clinical manifestation of the disease. Care providers may need to pay particular attention to the possible association of these two disorders since the co-occurrence of CVID and autoimmunity could be a misleading clue.
Objectives: Common variable immunodeficiency (CVID) is the most common symptomatic inborn error of immunity characterized by variable clinical manifestations. Methods: Web of Science, Scopus, and PubMed databases were searched systemically to find eligible studies from the earliest available date to February 2020 with standard keywords. Pooled estimates of the autoimmunity prevalence and the corresponding 95% confidence intervals (CI) were calculated using random-effects models. Results: The overall prevalence of autoimmunity was 29.8% (95% CI: 26.4-33.3; I2 = 82.8%). The prevalences of hematologic autoimmune diseases, autoimmune gastrointestinal disorders, autoimmune rheumatologic disorders, autoimmune skin disorders, and autoimmune endocrinopathy in CVIDpatients were 18.9%, 11.5%, 6.4%, 5.9%), and 2.5%, respectively. There were significantly higher lymphocyte, CD3 + T cell, and CD4 + T cell count among CVIDpatients without autoimmunity (p< 0.05). Furthermore, failure to thrive, organomegaly, enteropathy, and meningitis was significantly higher in CVIDpatients with autoimmunity(p< 0.05). Conclusions: Many CVIDpatients could present with autoimmunity as part of the disease or even as the first or only clinical manifestation of the disease. Care providers may need to pay particular attention to the possible association of these two disorders since the co-occurrence of CVID and autoimmunity could be a misleading clue.