{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,16]],"date-time":"2026-09-16T07:03:49Z","timestamp":1789542229847,"version":"build-2803163510"},"reference-count":36,"publisher":"Wiley","issue":"8","license":[{"start":{"date-parts":[[2026,8,23]],"date-time":"2026-08-23T00:00:00Z","timestamp":1787443200000},"content-version":"vor","delay-in-days":22,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2026,8,1]],"date-time":"2026-08-01T00:00:00Z","timestamp":1785542400000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Allergy Immunology"],"published-print":{"date-parts":[[2026,8]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Inborn errors of immunity (IEI) encompass over 600 disorders with diverse and overlapping presentations. Clinicians rely on pattern recognition\u2014clinical pearls\u2014to navigate this complexity, yet this experiential knowledge is rarely systematically compiled. Through a structured expert elicitation, we aimed to capture and organize clinical pearls, pitfalls, and caveats for the differential diagnosis of IEI as a practical educational reference for trainees and non\u2010specialist physicians. In a structured expert survey with narrative (thematic) synthesis\u2014not a formal consensus process\u2014twenty clinical immunologists were invited and twelve contributed (60% response). Each provided diagnostic pearls across six categories: red flags prompting IEI evaluation, signs suggestive of IEI categories, highly specific (near\u2010pathognomonic) findings, highly sensitive findings, expected phenotype (including exclusionary findings), and practical clinical wisdom. Responses were translated (Spanish to English where necessary), coded and synthesized by theme by two investigators with disagreements resolved by consensus, reviewed for contradictions, and supplemented with established pearls from the literature, with the origin of each pearl tracked. Contributors provided over 150 diagnostic pearls. High\u2010consensus red flags included disseminated BCG\/mycobacterial disease, very early\u2010onset inflammatory bowel disease (&lt;6\u2009years), and infant lymphopenia. Highly specific findings included microthrombocytopenia for Wiskott\u2010Aldrich syndrome, catalase\u2010positive organism infections for chronic granulomatous disease, and ataxia with telangiectasias for ataxia\u2010telangiectasia. Important caveats emerged: normal mean platelet volume does not exclude Wiskott\u2010Aldrich syndrome; normal IgG in older ataxia\u2010telangiectasia patients may mask antibody deficiency due to oligoclonal production; and absence of fever during serious bacterial infection suggests IRAK4\/MyD88 deficiency rather than arguing against it. Contributors emphasized that clinical phenotypes evolve over time and that patients may not fit established patterns at initial evaluation. This structured expert survey provides a practical, organized reference for IEI differential diagnosis. The compilation of pearls, pitfalls, and caveats, presented in eight tables, offers educational and hypothesis\u2010generating guidance for clinicians and trainees navigating these rare disorders. In an exploratory, qualitative comparison, several types of experiential clinical wisdom\u2014bedside decision rules, laboratory caveats, environmental guidance, and anticipatory management\u2014were underrepresented in the outputs of general\u2010purpose large language models informally queried on the same task, suggesting that expert\u2010derived knowledge of this kind may help inform future educational and diagnostic\u2010support efforts.<\/jats:p>\n                  <jats:p>\n                    <jats:boxed-text content-type=\"graphic\" position=\"anchor\">\n                      <jats:graphic xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" mimetype=\"image\/png\" position=\"anchor\" specific-use=\"enlarged-web-image\" xlink:href=\"graphic\/pai70470-gra-0001-m.png\">\n                        <jats:alt-text>image<\/jats:alt-text>\n                      <\/jats:graphic>\n                    <\/jats:boxed-text>\n                  <\/jats:p>","DOI":"10.1111\/pai.70470","type":"journal-article","created":{"date-parts":[[2026,8,24]],"date-time":"2026-08-24T05:56:40Z","timestamp":1787551000000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Clinical pearls and pitfalls in inborn errors of immunity: An expert\u2010derived framework for diagnostic pattern recognition"],"prefix":"10.1111","volume":"37","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-6747-5009","authenticated-orcid":false,"given":"Estefan\u00eda","family":"V\u00e1squez Echeverri","sequence":"first","affiliation":[{"name":"Facultad de Medicina Universidad EIA  Medell\u00edn Colombia"},{"name":"Unidad M\u00e9dica Quir\u00fargica Otorrinolaringolog\u00eda\u2010UNIMEQ\u2010ORL  Bogot\u00e1 Colombia"},{"name":"Inborn Errors of Immunity Group, Department of Microbiology and Parasitology, School of Medicine University of Antioquia UdeA  Medellin Colombia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3409-6226","authenticated-orcid":false,"given":"Juan Carlos","family":"Bustamante\u2010Ogando","sequence":"additional","affiliation":[{"name":"Clinical Immunology Service National Institute of Pediatrics, Health Secretariat  Mexico City Mexico"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1478-8401","authenticated-orcid":false,"given":"Lina M.","family":"Castano\u2010Jaramillo","sequence":"additional","affiliation":[{"name":"Pediatric Allergy and Clinical Immunology HOMI Hospital Pedi\u00e1trico la Misericordia  Bogot\u00e1 Colombia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2936-3679","authenticated-orcid":false,"given":"Natalia","family":"V\u00e9lez Tirado","sequence":"additional","affiliation":[{"name":"Pediatric Allergy and Clinical Immunology HOMI Hospital Pedi\u00e1trico la Misericordia  Bogot\u00e1 Colombia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7223-6651","authenticated-orcid":false,"given":"Edna","family":"Venegas Montoya","sequence":"additional","affiliation":[{"name":"Immunology Service, Hospital General Regional No. 1 Instituto Mexicano del Seguro Social (IMSS)  Culiac\u00e1n Sinaloa M\u00e9xico"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4849-2131","authenticated-orcid":false,"given":"H\u00e9ctor","family":"G\u00f3mez Tello","sequence":"additional","affiliation":[{"name":"Allergology Department, Hospital de la Ni\u00f1ez Poblana Servicios de Salud IMSS Bienestar  Puebla Puebla Mexico"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0007-0307-9737","authenticated-orcid":false,"given":"Alejandro","family":"Palma","sequence":"additional","affiliation":[{"name":"Division of Immunology, IWK Health Centre Dalhousie University  Halifax Nova Scotia Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6494-5511","authenticated-orcid":false,"given":"Eduardo","family":"Guan\u00ed\u2010Guerra","sequence":"additional","affiliation":[{"name":"Department of Medicine University of Guanajuato  Le\u00f3n Guanajuato Mexico"},{"name":"Department of Immunology Hospital Aranda de la Parra  Le\u00f3n Guanajuato M\u00e9xico"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0008-4583-1808","authenticated-orcid":false,"given":"Hanadys","family":"Ale","sequence":"additional","affiliation":[{"name":"Division of Immunology, Allergy, and Rheumatology Joe DiMaggio Children's Hospital, Memorial Healthcare System  Hollywood Florida USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7609-3923","authenticated-orcid":false,"given":"Marco 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