{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,3]],"date-time":"2026-06-03T12:04:53Z","timestamp":1780488293846,"version":"3.54.1"},"reference-count":19,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2025,5,26]],"date-time":"2025-05-26T00:00:00Z","timestamp":1748217600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Immunol."],"abstract":"<jats:p>STAT3-hyper IgE syndrome (STAT3-HIES) is a primary immunodeficiency disorder caused by dominant-negative mutations in <jats:italic>STAT3<\/jats:italic>, leading to defects in Th17 cell differentiation, immune regulation, and tissue repair. Patients are susceptible to recurrent infections and vascular abnormalities, such as vasculopathy and pseudoaneurysms. While involvement of cerebral, bronchial, and coronary arteries has been reported, hepatic artery involvement is rare. We describe a 25-year-old woman with genetically confirmed STAT3-HIES who presented with biliary hemorrhage secondary to a ruptured hepatic pseudoaneurysm. Emergency transcatheter arterial embolization successfully controlled the hemorrhage, and the patient was discharged without complications. Systemic vascular screening revealed an asymptomatic right coronary artery dilation, necessitating medical management with statin therapy. This case highlights hepatic pseudoaneurysm as a rare but life-threatening vascular complication in STAT3-HIES. Given the potential for multi-organ vasculopathy, systemic vascular screening by contrast-enhanced CT or MRI is crucial for early detection and management. Further research is needed to elucidate the mechanisms underlying vasculopathy in STAT3-HIES and establish optimal screening strategies to improve patient outcomes.<\/jats:p>","DOI":"10.3389\/fimmu.2025.1601776","type":"journal-article","created":{"date-parts":[[2025,5,26]],"date-time":"2025-05-26T05:17:35Z","timestamp":1748236655000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":2,"title":["Case Report: Biliary hemorrhage by intrahepatic pseudoaneurysm and asymptomatic right coronary artery pseudoaneurysm in a patient with STAT3 hyper IgE syndrome"],"prefix":"10.3389","volume":"16","author":[{"given":"Daiki","family":"Fujita","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Masahiro","family":"Ueki","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hiroshi","family":"Yamanaka","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kota","family":"Watanabe","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Satoshi","family":"Yakuwa","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Atsushi","family":"Manabe","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Masafumi","family":"Yamada","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2025,5,26]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","DOI":"10.1007\/s10875-022-01289-3","article-title":"Human inborn errors of immunity: 2022 update on the classification from the international union of immunological societies expert committee","volume":"42","author":"Tangye","year":"2022","journal-title":"J Clin Immunol"},{"key":"B2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa073687","article-title":"STAT3 mutations in the hyper-IgE syndrome","volume":"357","author":"Holland","year":"2007","journal-title":"New Engl J Med"},{"key":"B3","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2249.2009.04044.x","article-title":"A review on the vascular features of the hyperimmunoglobulin E syndrome","volume":"159","author":"Yavuz","year":"2010","journal-title":"Clin Exp Immunol"},{"key":"B4","doi-asserted-by":"publisher","first-page":"25","DOI":"10.1161\/CIRCGENETICS.111.961235","article-title":"Frequent and widespread vascular abnormalities in human signal transducer and activator of transcription 3 deficiency","volume":"5","author":"Chandesris","year":"2012","journal-title":"Circ Cardiovasc Genet"},{"key":"B5","doi-asserted-by":"publisher","DOI":"10.1084\/jem.20100799","article-title":"Defective IL-10 signaling in hyper-IgE syndrome results in impaired generation of tolerogenic dendritic cells and induced regulatory T cells","volume":"208","author":"Saito","year":"2011","journal-title":"J Exp Med"},{"key":"B6","doi-asserted-by":"publisher","DOI":"10.1007\/BF02012742","article-title":"Bronchial artery aneurysm in hyperimmunoglobulinemia E syndrome","volume":"24","author":"Connolly","year":"1994","journal-title":"Pediatr Radiol"},{"key":"B7","doi-asserted-by":"publisher","DOI":"10.1016\/j.clim.2006.10.005","article-title":"Coronary artery aneurysms in patients with hyper IgE recurrent infection syndrome","volume":"122","author":"Ling","year":"2007","journal-title":"Clin Immunol (Orlando Fla)"},{"key":"B8","doi-asserted-by":"publisher","DOI":"10.1155\/2009\/586919","article-title":"Recurrent hemoptysis with Penicillium marneffei and Stenotrophomonas maltophilia in Job\u2019s syndrome","volume":"16","author":"Ma","year":"2009","journal-title":"Can Respir J"},{"key":"B9","doi-asserted-by":"publisher","DOI":"10.1007\/s10875-010-9479-1","article-title":"Clinical aspects and genetic analysis of Taiwanese patients with the phenotype of hyper-immunoglobulin E recurrent infection syndromes (HIES)","volume":"31","author":"Lee","year":"2011","journal-title":"J Clin Immunol"},{"key":"B10","doi-asserted-by":"publisher","DOI":"10.1016\/j.jvs.2011.08.011","article-title":"Ruptured thoracoabdominal aneurysm in a 27-year-old with hyper IgE syndrome","volume":"55","author":"Falah","year":"2012","journal-title":"J Vasc Surg"},{"key":"B11","doi-asserted-by":"publisher","DOI":"10.1016\/j.anai.2015.02.016","article-title":"Cerebral aneurysm in a 12-year-old boy with a STAT3 mutation (hyper-IgE syndrome)","volume":"114","author":"Kim","year":"2015","journal-title":"Ann Allergy Asthma Immunol"},{"key":"B12","doi-asserted-by":"publisher","first-page":"34","DOI":"10.1186\/s12890-019-0797-7","article-title":"Pulmonary artery pseudoaneurysm causing massive hemoptysis in hyperimmunoglobulin E syndrome: a case report","volume":"19","author":"Hakim","year":"2019","journal-title":"BMC Pulm Med"},{"key":"B13","doi-asserted-by":"publisher","DOI":"10.1177\/1591019919828657","article-title":"Delayed development of a de novo contralateral middle cerebral artery aneurysm in a patient with hyperimmunoglobulin E syndrome: A case report","volume":"25","author":"Nussbaum","year":"2019","journal-title":"Interv Neuroradiol"},{"key":"B14","doi-asserted-by":"publisher","DOI":"10.3389\/fped.2020.00575","article-title":"Hematopoietic stem cell transplantation and vasculopathy associated with STAT3-dominant-negative hyper-igE syndrome","volume":"8","author":"Ponsford","year":"2020","journal-title":"Front Pediatr"},{"key":"B15","doi-asserted-by":"publisher","DOI":"10.3390\/antibiotics13090866","article-title":"Efficacy and safety of antibiotics in the treatment of methicillin-resistant staphylococcus aureus (MRSA) infections: A systematic review and network meta-analysis","volume":"13","author":"Liu","year":"2024","journal-title":"Antibiot (Basel)"},{"key":"B16","doi-asserted-by":"publisher","DOI":"10.1172\/JCI135490","article-title":"Impaired angiogenesis and extracellular matrix metabolism in autosomal-dominant hyper-IgE syndrome","volume":"130","author":"Dmitrieva","year":"2020","journal-title":"J Clin Invest"},{"key":"B17","doi-asserted-by":"publisher","DOI":"10.1007\/s10875-021-01051-1","article-title":"STAT3 hyper-igE syndrome-an update and unanswered questions","volume":"41","author":"Tsilifis","year":"2021","journal-title":"J Clin Immunol"},{"key":"B18","doi-asserted-by":"publisher","DOI":"10.1016\/j.atherosclerosis.2017.01.022","article-title":"Coronary atherosclerosis and dilation in hyper IgE syndrome patients: Depiction by magnetic resonance vessel wall imaging and pathological correlation","volume":"258","author":"Abd-Elmoniem","year":"2017","journal-title":"Atherosclerosis"},{"key":"B19","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1016\/j.phrs.2016.02.012","article-title":"Rosuvastatin: Beyond the cholesterol-lowering effect","volume":"107","author":"Cortese","year":"2016","journal-title":"Pharmacol Res"}],"container-title":["Frontiers in Immunology"],"original-title":[],"link":[{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fimmu.2025.1601776\/full","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,5,26]],"date-time":"2025-05-26T05:17:36Z","timestamp":1748236656000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fimmu.2025.1601776\/full"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,5,26]]},"references-count":19,"alternative-id":["10.3389\/fimmu.2025.1601776"],"URL":"https:\/\/doi.org\/10.3389\/fimmu.2025.1601776","relation":{},"ISSN":["1664-3224"],"issn-type":[{"value":"1664-3224","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,5,26]]},"article-number":"1601776"}}