{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,1]],"date-time":"2026-09-01T06:13:33Z","timestamp":1788243213507,"version":"build-2803163510"},"reference-count":18,"publisher":"American Academy of Pediatrics (AAP)","issue":"5","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2026,5,1]]},"abstract":"<jats:p>A 4-year-old child presented with a recent history of loss of appetite, weight loss, lower limb myalgias, fatigue, and new-onset hearing loss. These symptoms were associated with persistent microcytic anemia, leukocytosis, thrombocytosis, hypergammaglobulinemia, and elevated erythrocyte sedimentation rate. Major oncological, hematological, gastrointestinal, rheumatological, immunological, and infectious conditions were ruled out.<\/jats:p>\n                  <jats:p>Suspecting an autoinflammatory disease, high-dose corticosteroids were initiated but without clinical improvement. Due to the severe inflammatory state in the absence of autoimmune or infectious causes, anakinra was started, resulting in only transient benefit. The patient subsequently developed uveitis, and Cogan syndrome (CS) was suspected. Infliximab was administered but with no improvement. Treatment was then switched to tocilizumab, which led to both clinical and laboratory improvement.<\/jats:p>\n                  <jats:p>After 1\u00a0year of good disease control, the patient unexpectedly died. Autopsy revealed extensive involvement of the coronary and aortic vessels, with polymorphic inflammatory infiltrates.<\/jats:p>\n                  <jats:p>Cogan syndrome is a rare vasculitis that can affect vessels of all sizes, typically involving both the ocular and cochleovestibular systems. It is extremely rare in children, with only about 50 cases reported and just 2 deaths previously described.<\/jats:p>\n                  <jats:p>Unfortunately, there are no international guidelines for the treatment and follow-up of pediatric patients with CS. This case represents an uncommon manifestation of CS in a child and highlights the urgent need for evidence-based guidelines for the diagnosis, management, and long-term monitoring of these patients.<\/jats:p>","DOI":"10.1542\/peds.2025-073105","type":"journal-article","created":{"date-parts":[[2026,4,15]],"date-time":"2026-04-15T00:02:50Z","timestamp":1776211370000},"source":"Crossref","is-referenced-by-count":1,"title":["Unexpected Death in a Young Child With Cogan Syndrome"],"prefix":"10.1542","volume":"157","author":[{"given":"Serena","family":"Pastore","sequence":"first","affiliation":[{"name":"1Institute for Maternal and Child Health, IRCCS Burlo Garofolo, Trieste, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francesca","family":"Burlo","sequence":"additional","affiliation":[{"name":"2Department of Medicine, Surgery, and Health Sciences, University of Trieste, Trieste, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrea","family":"Taddio","sequence":"additional","affiliation":[{"name":"1Institute for Maternal and Child Health, IRCCS Burlo Garofolo, Trieste, Italy"},{"name":"2Department of Medicine, Surgery, and Health Sciences, University of Trieste, Trieste, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giulia","family":"Gortani","sequence":"additional","affiliation":[{"name":"1Institute for Maternal and Child Health, IRCCS Burlo Garofolo, Trieste, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alberto","family":"Tommasini","sequence":"additional","affiliation":[{"name":"1Institute for Maternal and Child Health, IRCCS Burlo Garofolo, Trieste, Italy"},{"name":"2Department of Medicine, Surgery, and Health Sciences, University of Trieste, Trieste, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daniela","family":"Chicco","sequence":"additional","affiliation":[{"name":"1Institute for Maternal and Child Health, IRCCS Burlo Garofolo, Trieste, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cristina","family":"Basso","sequence":"additional","affiliation":[{"name":"3Cardiovascular Pathology Unit, Department of Cardiac, Thoracic, Vascular Sciences and Public Health, University of Padua, Azienda Ospedaliera, Padova, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Monica","family":"De Gaspari","sequence":"additional","affiliation":[{"name":"3Cardiovascular Pathology Unit, Department of Cardiac, Thoracic, Vascular Sciences and Public Health, University of Padua, Azienda Ospedaliera, Padova, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"417","published-online":{"date-parts":[[2026,4,15]]},"reference":[{"issue":"7","key":"2026090101171086700_r1","doi-asserted-by":"crossref","first-page":"1102","DOI":"10.1002\/art.42139","article-title":"The 2021 EULAR\/American College of Rheumatology points to consider for diagnosis, management and monitoring of the interleukin-1 mediated autoinflammatory diseases: cryopyrin-associated periodic syndromes, tumour necrosis factor receptor-associated periodic syndrome, mevalonate kinase deficiency, and deficiency of the interleukin-1 receptor antagonist","volume":"74","author":"Romano","year":"2022","journal-title":"Arthritis Rheumatol."},{"issue":"9","key":"2026090101171086700_r2","doi-asserted-by":"crossref","first-page":"865","DOI":"10.1111\/jpc.13326","article-title":"Periodic fevers and autoinflammatory syndromes in childhood","volume":"52","author":"Ostring","year":"2016","journal-title":"J Paediatr Child Health."},{"issue":"1","key":"2026090101171086700_r3","doi-asserted-by":"crossref","first-page":"167","DOI":"10.1186\/s13023-016-0542-8","article-title":"Chronic infantile neurological cutaneous and articular (CINCA) syndrome: a review","volume":"11","author":"Finetti","year":"2016","journal-title":"Orphanet J Rare Dis."},{"issue":"1","key":"2026090101171086700_r4","doi-asserted-by":"crossref","first-page":"54","DOI":"10.1186\/s12969-023-00830-x","article-title":"Paediatric Cogan\u00b4s syndrome - 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