autoimmunity complications in common variable immunodeficiency patients; an update from the iranian registry

Background/objectives: cvid is known as the most prevalent symptomatic primary immunodeficiency(pid) characterized by heterozygous manifestations including several infectious and non-infectiouscomplications. accordingly, although the hallmark of this disease is the development ofrecurrent sinopulmonary infections, different types of autoimmunity are frequently reported in cvidsubjects. so in this study, we aimed to provide an update report on various autoimmunity manifestationsin a group of cvid patients in iran.methods: demographic, clinical, and immunologic data of iranian cvid cases who were followedup at children’s medical center were collected. based on the presence of autoimmunity, the patientswere 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 mostlymanifested as autoimmune cytopenia; 21 (24%) out of these 81 individuals had immune thrombocytopenicpurpura (itp) and 14 (17.3%) patients showed autoimmune hemolytic anemia (aiha).moreover, rheumatologic autoimmune disorders such as juvenile idiopathic arthritis (jia) and juvenilerheumatoid 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 mostobserved gastrointestinal and dermatologic autoimmune disorders in this study. accordingly, someof these conditions were concomitant in a single individual. additionally, several significant correlationswere 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.