{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,24]],"date-time":"2026-01-24T07:16:45Z","timestamp":1769239005799,"version":"3.49.0"},"reference-count":10,"publisher":"Wiley","issue":"S26","license":[{"start":{"date-parts":[[2020,11,24]],"date-time":"2020-11-24T00:00:00Z","timestamp":1606176000000},"content-version":"vor","delay-in-days":23,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Allergy Immunology"],"published-print":{"date-parts":[[2020,11]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Common variable immunodeficiency (CVID) is the most prevalent primary immune deficiency, affecting roughly 2\u20104\/100\u00a0000 individuals in the general population. The etiology is currently unknown, even if several genetic mutations have been described, and no single clinical feature or single laboratory test can establish the diagnosis, which is based on multiple criteria. CVID is characterized by B\u2010 and T\u2010cell dysfunction that predisposes to an increased risk of infections, with the typical involvement of the respiratory and gastrointestinal tracts. Besides this well\u2010established complication, though the coexistence of immunodeficiency and autoimmunity appears paradoxical, two\u2010thirds of CVID patients present concomitant autoimmune disorders. Of note, autoimmunity can often be the only clinical manifestation of CVID at the time of diagnosis. The most common autoimmune disorders associated with CVID are autoimmune cytopenias, that is, immune thrombocytopenic purpura and autoimmune hemolytic anemia, either as separate entities or as concurrently (Evans syndrome). CVID patients can also manifest rheumatologic diseases (eg, arthritis, Sjogren's disease, systemic lupus erythematosus, vasculitis, Beh\u00e7et's syndrome) and gastrointestinal autoimmune disorders (eg, ulcerative colitis, autoimmune atrophic gastritis, celiac disease). This latter may pose a particular diagnostic challenge, as celiac\u2010specific autoantibodies can be absent in CVID patients. Understanding the molecular basis and the clinical impact of autoimmunity in CVID patients might help manage CVID, thus reducing its diagnostic delay and preventing its complications.<\/jats:p>","DOI":"10.1111\/pai.13339","type":"journal-article","created":{"date-parts":[[2020,11,25]],"date-time":"2020-11-25T06:36:06Z","timestamp":1606286166000},"page":"60-62","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":11,"title":["Autoimmune diseases associated with common variable immune deficiency"],"prefix":"10.1111","volume":"31","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6654-4911","authenticated-orcid":false,"given":"Marco Vincenzo","family":"Lenti","sequence":"first","affiliation":[{"name":"Department of Internal Medicine San Matteo Hospital Foundation University of Pavia  Pavia Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jessica","family":"Savioli","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine San Matteo Hospital Foundation University of Pavia  Pavia Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Giovanna","family":"Achilli","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine San Matteo Hospital Foundation University of Pavia  Pavia Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0302-8645","authenticated-orcid":false,"given":"Antonio","family":"Di Sabatino","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine San Matteo Hospital Foundation University of Pavia  Pavia Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2020,11,24]]},"reference":[{"key":"e_1_2_6_2_1","doi-asserted-by":"publisher","DOI":"10.1006\/clim.1999.4799"},{"key":"e_1_2_6_3_1","doi-asserted-by":"publisher","DOI":"10.1111\/cei.12178"},{"key":"e_1_2_6_4_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10875-007-9075-1"},{"key":"e_1_2_6_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaut.2010.10.002"},{"key":"e_1_2_6_6_1","doi-asserted-by":"publisher","DOI":"10.1186\/s13023-018-0941-0"},{"key":"e_1_2_6_7_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10875-017-0456-9"},{"key":"e_1_2_6_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.semarthrit.2018.02.013"},{"key":"e_1_2_6_9_1","doi-asserted-by":"publisher","DOI":"10.1111\/apt.15317"},{"key":"e_1_2_6_10_1","doi-asserted-by":"publisher","DOI":"10.1309\/AJCPEIILH2C0WFYE"},{"key":"e_1_2_6_11_1","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2019.02937"}],"container-title":["Pediatric Allergy and Immunology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/pai.13339","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1111\/pai.13339","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/pai.13339","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,2]],"date-time":"2023-09-02T19:16:35Z","timestamp":1693682195000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/pai.13339"}},"subtitle":[],"editor":[{"given":"Philippe","family":"Eigenmann","sequence":"additional","affiliation":[],"role":[{"role":"editor","vocabulary":"crossref"}]}],"short-title":[],"issued":{"date-parts":[[2020,11]]},"references-count":10,"journal-issue":{"issue":"S26","published-print":{"date-parts":[[2020,11]]}},"alternative-id":["10.1111\/pai.13339"],"URL":"https:\/\/doi.org\/10.1111\/pai.13339","archive":["Portico"],"relation":{},"ISSN":["0905-6157","1399-3038"],"issn-type":[{"value":"0905-6157","type":"print"},{"value":"1399-3038","type":"electronic"}],"subject":[],"published":{"date-parts":[[2020,11]]},"assertion":[{"value":"2020-07-29","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2020-08-11","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2020-11-24","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}