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Clinically, <jats:styled-content style=\"fixed-case\">CVID<\/jats:styled-content> is characterized by increased susceptibility to infections and a wide variety of autoimmune and rheumatologic disorders. All patients with <jats:styled-content style=\"fixed-case\">CVID<\/jats:styled-content> registered in Iranian <jats:styled-content style=\"fixed-case\">PID<\/jats:styled-content> Registry (<jats:styled-content style=\"fixed-case\">IPIDR<\/jats:styled-content>) were enrolled in this retrospective cohort study. We investigated the frequency of rheumatologic diseases and its association with immunological and clinical phenotypes in patients with <jats:styled-content style=\"fixed-case\">CVID<\/jats:styled-content>. A total of 227 patients with <jats:styled-content style=\"fixed-case\">CVID<\/jats:styled-content> were enrolled in this study. The prevalence of rheumatologic disorders was 10.1% with a higher frequency in women than men. Most common rheumatologic manifestations were juvenile idiopathic arthritis (<jats:styled-content style=\"fixed-case\">JIA<\/jats:styled-content>) and adult rheumatoid arthritis (<jats:styled-content style=\"fixed-case\">RA<\/jats:styled-content>) followed by juvenile spondyloarthritis (<jats:styled-content style=\"fixed-case\">JS<\/jats:styled-content>pA) and undifferentiated inflammatory arthritis (<jats:styled-content style=\"fixed-case\">UIA<\/jats:styled-content>). Septic arthritis in patients with <jats:styled-content style=\"fixed-case\">CVID<\/jats:styled-content> with a history of <jats:styled-content style=\"fixed-case\">RA<\/jats:styled-content> and <jats:styled-content style=\"fixed-case\">JIA<\/jats:styled-content> was higher than patients without rheumatologic complication. Patients with <jats:styled-content style=\"fixed-case\">CVID<\/jats:styled-content> with a history of autoimmunity (both rheumatologic and non\u2010rheumatologic autoimmunity) had lower regulatory T cells counts in comparison with patients without autoimmune disorders. There was an association between defect in specific antibody responses and negative serologic test results in patients with rheumatologic manifestations. <jats:styled-content style=\"fixed-case\">JIA<\/jats:styled-content>,<jats:styled-content style=\"fixed-case\"> RA<\/jats:styled-content>,<jats:styled-content style=\"fixed-case\"> JS<\/jats:styled-content>pA and <jats:styled-content style=\"fixed-case\">UIA<\/jats:styled-content> are the most frequent rheumatologic disorders in patients with <jats:styled-content style=\"fixed-case\">CVID<\/jats:styled-content>. Due to antibody deficiency, serologic tests may be negative in these patients. Therefore, these conditions pose significant diagnostic and therapeutic challenges for immunologists and rheumatologists in charge of the care for these patients.<\/jats:p>","DOI":"10.1111\/sji.12663","type":"journal-article","created":{"date-parts":[[2018,3,25]],"date-time":"2018-03-25T06:53:52Z","timestamp":1521960832000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":30,"title":["Rheumatologic complications in a cohort of 227 patients with common variable immunodeficiency"],"prefix":"10.1111","volume":"87","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-5658-2511","authenticated-orcid":false,"given":"G.","family":"Azizi","sequence":"first","affiliation":[{"name":"Non\u2010Communicable Diseases Research Center Alborz University of Medical Sciences Karaj Iran"},{"name":"Department of Laboratory Medicine Imam Hassan Mojtaba Hospital Alborz University of Medical Sciences Karaj Iran"},{"name":"Research Center for 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