{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,7]],"date-time":"2026-04-07T05:12:06Z","timestamp":1775538726626,"version":"3.50.1"},"reference-count":29,"publisher":"Oceanside Publications Inc.","issue":"2","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["allergy asthma proc"],"published-print":{"date-parts":[[2026,3,1]]},"abstract":"<jats:p>\n                    <jats:bold>Background:<\/jats:bold>\n                    Autoimmunity and autoinflammation are increasingly recognized manifestations of immune dysregulation in patients with primary immunodeficiency (PID), alongside recurrent infections.\n                    <jats:bold>Objective:<\/jats:bold>\n                    The objectives were to determine the prevalence\n of autoimmune and autoinflammatory findings in patients with PID and to compare their clinical, laboratory, and immunologic characteristics.\n                    <jats:bold>Methods:<\/jats:bold>\n                    This retrospective study included 130 adult patients with PID who were followed up at a tertiary university immunology clinic.\n The patients were divided into three groups: those without immune dysregulation (group 1), those with predominant autoimmune features (group 2), and those with predominant autoinflammatory features (group 3). Demographic data, laboratory parameters, immunoglobulin levels, lymphocyte subsets,\n switched memory B cells, and genetic mutation data were analyzed.\n                    <jats:bold>Results:<\/jats:bold>\n                    Autoimmune manifestations were predominant in 32.3% and autoinflammatory findings were predominant in 9.2% of the patients. The patients in group 3 showed significantly elevated C-reactive protein,\n erythrocyte sedimentation rate (ESR), and fibrinogen levels (p &lt;\u20090.05). Of the patients in group 2, the platelet count, serum immunoglobulin E (IgE) level, and switched memory B cells were significantly lower compared with other groups (p =\u20090.001, p =\u20090.007, p =\u20090.005,\n respectively). There were no significant differences in genetic mutation frequency or mortality among groups.\n                    <jats:bold>Conclusion:<\/jats:bold>\n                    Autoimmunity and autoinflammation are frequent in PID and associated with distinct immunologic profiles. Elevated acute phase reactants may indicate\n autoinflammation, whereas low IgE levels and decreased switched memory B cells may serve as early markers of autoimmunity. Routine evaluation for immune dysregulation in patients with PID is recommended.\n                  <\/jats:p>","DOI":"10.2500\/aap.2026.47.250102","type":"journal-article","created":{"date-parts":[[2026,3,17]],"date-time":"2026-03-17T04:35:38Z","timestamp":1773722138000},"page":"149-156","source":"Crossref","is-referenced-by-count":1,"title":["Comparison of clinical characteristics in primary immunodeficiency patients based on the presence of autoimmunity or autoinflammation"],"prefix":"10.2500","volume":"47","author":[{"given":"Se\u00e7im","family":"Kolak","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Fatih","family":"\u00c7\u00f6lkesen","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mehmet Emin","family":"Gerek","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ferhat","family":"Sa\u011fun","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Emrah","family":"Harman","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"\u015e\u00fckran Aslan","family":"Sava\u015f","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"\u015eevket","family":"Arslan","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1355","reference":[{"key":"B1","doi-asserted-by":"crossref","DOI":"10.70962\/jhi.20250003","article-title":"Human inborn errors of immunity: 2024 update on the classification from the International Union of Immunological Societies Expert Committee","volume":"1","author":"Poli","year":"2025","journal-title":"J Hum Immun"},{"key":"B2","doi-asserted-by":"crossref","first-page":"1704","DOI":"10.1016\/j.jaci.2024.01.011","article-title":"Validating inborn error of immunity prevalence and risk with nationally representative electronic health record data","volume":"153","author":"Rider","year":"2024","journal-title":"J Allergy Clin Immunol"},{"key":"B3","doi-asserted-by":"crossref","first-page":"157","DOI":"10.2174\/1871530319666190828125316","article-title":"Diagnostic approach to the patients with suspected primary \u0131mmunodeficiency","volume":"20","author":"Tavakol","year":"2020","journal-title":"Endocr Metab Immune Disord Drug Targets"},{"key":"B4","doi-asserted-by":"crossref","first-page":"989","DOI":"10.1080\/1744666X.2016.1177458","article-title":"Autoimmunity in primary T-cell immunodeficiencies","volume":"12","author":"Azizi","year":"2016","journal-title":"Expert Rev Clin Immunol"},{"key":"B5","doi-asserted-by":"crossref","first-page":"764","DOI":"10.1007\/s10875-016-0330-1","article-title":"Autoimmune and \u0131nflammatory manifestations in 247 patients with primary \u0131mmunodeficiency-a report from the Slovenian National Registry","volume":"36","author":"Blazina","year":"2016","journal-title":"J Clin Immunol"},{"key":"B6","doi-asserted-by":"crossref","first-page":"1388","DOI":"10.1016\/j.jaci.2016.12.978","article-title":"Autoimmune and inflammatory manifestations occur frequently in patients with primary immunodeficiencies","volume":"140","author":"Fischer","year":"2017","journal-title":"J Allergy Clin Immunol"},{"key":"B7","doi-asserted-by":"crossref","first-page":"4729","DOI":"10.3390\/jcm10204729","article-title":"Autoimmunity in primary \u0131mmunodeficiency disorders: an updated review on pathogenic and clinical \u0131mplications","volume":"10","author":"Costagliola","year":"2021","journal-title":"J Clin Med"},{"key":"B8","doi-asserted-by":"crossref","first-page":"471","DOI":"10.1016\/j.det.2013.05.001","article-title":"Autoinflammatory syndromes","volume":"31","author":"Cush","year":"2013","journal-title":"Dermatol Clin"},{"key":"B9","doi-asserted-by":"crossref","first-page":"210","DOI":"10.3389\/fimmu.2012.00210","article-title":"Pathogenesis of autoimmunity in common variable immunodeficiency","volume":"3","author":"Warnatz","year":"2012","journal-title":"Front Immunol"},{"key":"B10","doi-asserted-by":"crossref","first-page":"116","DOI":"10.1016\/j.jaci.2013.12.1077","article-title":"Clinical picture and treatment of 2212 patients with common variable immunodeficiency","volume":"134","author":"Gathmann","year":"2014","journal-title":"J Allergy Clin Immunol"},{"key":"B11","doi-asserted-by":"crossref","first-page":"1763","DOI":"10.1016\/j.jaip.2019.02.004","article-title":"The European Society for Immunodeficiencies (ESID) Registry Working Definitions for the clinical diagnosis of inborn errors of immunity","volume":"7","author":"Seidel","year":"2019","journal-title":"J Allergy Clin Immunol Pract"},{"key":"B12","doi-asserted-by":"crossref","first-page":"295","DOI":"10.1093\/cei\/uxac098","article-title":"Autoinflammatory manifestations in adult patients","volume":"210","author":"Rodolfi","year":"2022","journal-title":"Clin Exp Immunol"},{"key":"B13","doi-asserted-by":"crossref","first-page":"242","DOI":"10.1007\/s12098-015-1985-y","article-title":"Autoinflammatory syndromes in children","volume":"83","author":"Pepper","year":"2016","journal-title":"Indian J Pediatr"},{"key":"B14","doi-asserted-by":"crossref","first-page":"185","DOI":"10.1186\/1741-7015-11-185","article-title":"The hyperferritinemic syndrome: macrophage activation syndrome, Still's disease, septic shock and catastrophic antiphospholipid syndrome","volume":"11","author":"Ros\u00e1rio","year":"2013","journal-title":"BMC Med"},{"key":"B15","doi-asserted-by":"crossref","first-page":"135","DOI":"10.1038\/nrrheum.2013.174","article-title":"A clinical guide to autoinflammatory diseases: familial Mediterranean fever and next-of-kin","volume":"10","author":"Ozen","year":"2014","journal-title":"Nat Rev Rheumatol"},{"key":"B16","doi-asserted-by":"crossref","first-page":"25","DOI":"10.1016\/j.jaut.2010.10.002","article-title":"Autoimmunity in common variable immunodeficiency: correlation with lymphocyte phenotype in the French DEFI study","volume":"36","author":"Boileau","year":"2011","journal-title":"J Autoimmun"},{"key":"B17","doi-asserted-by":"crossref","first-page":"e91","DOI":"10.2500\/aap.2025.46.250016","article-title":"Selective immunoglobulin E deficiency and its association with autoimmune and autoinflammatory diseases","volume":"46","author":"Gerek","year":"2025","journal-title":"Allergy Asthma Proc"},{"key":"B18","doi-asserted-by":"crossref","first-page":"e11","DOI":"10.2500\/aap.2023.44.230025","article-title":"Relationship between autoimmune diseases and serum basal immunoglobulin E levels in patients with common variable immunodeficiency","volume":"44","author":"K\u0131l\u0131nc","year":"2023","journal-title":"Allergy Asthma Proc"},{"key":"B19","doi-asserted-by":"crossref","first-page":"e27\u2013e33","DOI":"10.2500\/aap.2014.35.3734","article-title":"Selective IgE deficiency, immune dysregulation, and autoimmunity","volume":"35","author":"Magen","year":"2014","journal-title":"Allergy Asthma Proc"},{"key":"B20","doi-asserted-by":"crossref","first-page":"313","DOI":"10.1016\/S1081-1206(10)63188-2","article-title":"Clinical manifestations of IgE hypogammaglobulinemia","volume":"78","author":"Smith","year":"1997","journal-title":"Ann Allergy Asthma Immunol"},{"key":"B21","doi-asserted-by":"crossref","first-page":"4160","DOI":"10.3390\/jcm10184160","article-title":"Spectrum of disease manifestations in patients with selective \u0131mmunoglobulin E deficiency","volume":"10","author":"Picado","year":"2021","journal-title":"J Clin Med"},{"key":"B22","doi-asserted-by":"crossref","first-page":"918","DOI":"10.16899\/jcm.1350196","article-title":"Relationship of selective IgE deficiency with autoimmune diseases","volume":"13","author":"Sayaca","year":"2023","journal-title":"J Contemp Med"},{"key":"B23","doi-asserted-by":"crossref","first-page":"1893","DOI":"10.1007\/s10875-021-01127-y","article-title":"Is there a clinical significance of very low serum \u0131mmunoglobulin E level?","volume":"41","author":"Al","year":"2021","journal-title":"J Clin Immunol"},{"key":"B24","doi-asserted-by":"crossref","first-page":"e38397","DOI":"10.1097\/MD.0000000000038397","article-title":"Various clinical manifestations of 223 patients with IgE deficiency in a tertiary hospital in China: a cross-sectional study","volume":"103","author":"Zhang","year":"2024","journal-title":"Medicine (Baltimore)"},{"key":"B25","doi-asserted-by":"crossref","first-page":"3356","DOI":"10.3390\/jcm10153356","article-title":"Patients with common variable \u0131mmunodeficiency complicated by autoimmune phenomena have lymphopenia and reduced Treg, Th17, and NK cells","volume":"10","author":"Wi\u0119sik-Szewczyk","year":"2021","journal-title":"J Clin Med"},{"key":"B26","doi-asserted-by":"crossref","first-page":"112446","DOI":"10.1016\/j.celrep.2023.112446","article-title":"Defective peripheral B cell selection in common variable immune deficiency patients with autoimmune manifestations","volume":"42","author":"Friman","year":"2023","journal-title":"Cell Rep"},{"key":"B27","doi-asserted-by":"crossref","first-page":"746","DOI":"10.1007\/s10875-010-9424-3","article-title":"B-cell and T-cell phenotypes in CVID patients correlate with the clinical phenotype of the disease","volume":"30","author":"Mouillot","year":"2010","journal-title":"J Clin Immunol"},{"key":"B28","doi-asserted-by":"crossref","first-page":"1544","DOI":"10.1182\/blood.V99.5.1544","article-title":"Severe deficiency of switched memory B cells (CD27(+)IgM(-)IgD(-)) in subgroups of patients with common variable immunodeficiency: a new approach to classify a heterogeneous disease","volume":"99","author":"Warnatz","year":"2002","journal-title":"Blood"},{"key":"B29","doi-asserted-by":"crossref","first-page":"822400","DOI":"10.3389\/fped.2022.822400","article-title":"CD21\u2013 CD27\u2013 atypical B cells in a pediatric cohort study: an extensive single center flow cytometric analysis","volume":"10","author":"Corrente","year":"2022","journal-title":"Front Pediatr"}],"container-title":["Allergy and Asthma Proceedings"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.ingentaconnect.com\/content\/ocean\/aap\/2026\/00000047\/00000002\/art00012","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,4,7]],"date-time":"2026-04-07T04:30:51Z","timestamp":1775536251000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.ingentaconnect.com\/content\/10.2500\/aap.2026.47.250102"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,3,1]]},"references-count":29,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2026,3,1]]}},"alternative-id":["1088-5412(20260301)47:2L.149;1-"],"URL":"https:\/\/doi.org\/10.2500\/aap.2026.47.250102","relation":{},"ISSN":["1088-5412"],"issn-type":[{"value":"1088-5412","type":"print"}],"subject":[],"published":{"date-parts":[[2026,3,1]]}}}