Ambre Dethier, Fanny Bajolle, Paul Bastard, Laureline Berteloot, Romain Lévy, Filippo Consonni, Quentin Deletang, Eric Dannaoui, Olivier Raisky, Philippe Drabent, Jacinta Bustamante, Fréderic Rieux-Laucat, Anne Puel, Fanny Lanternier, Marie-Louise Frémond, Bénédicte Neven
{"title":"A fatal case of Aspergillus endocarditis in a pediatric patient with STING-associated vasculopathy.","authors":"Ambre Dethier, Fanny Bajolle, Paul Bastard, Laureline Berteloot, Romain Lévy, Filippo Consonni, Quentin Deletang, Eric Dannaoui, Olivier Raisky, Philippe Drabent, Jacinta Bustamante, Fréderic Rieux-Laucat, Anne Puel, Fanny Lanternier, Marie-Louise Frémond, Bénédicte Neven","doi":"10.70962/jhi.20250262","DOIUrl":null,"url":null,"abstract":"<p><p>SAVI is a rare STING1 gain-of-function disorder causing systemic inflammation, vasculopathy, and interstitial lung disease. JAK inhibitors are first-line therapy. We report a SAVI patient on prolonged JAK inhibitors who developed opportunistic infections, including fatal Aspergillus endocarditis, underscoring therapeutic challenges.</p>","PeriodicalId":521015,"journal":{"name":"Journal of human immunity","volume":"2 6","pages":""},"PeriodicalIF":0.0000,"publicationDate":"2026-11-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13596074/pdf/","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Journal of human immunity","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.70962/jhi.20250262","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2026/9/22 0:00:00","PubModel":"Epub","JCR":"","JCRName":"","Score":null,"Total":0}
引用次数: 0
Abstract
SAVI is a rare STING1 gain-of-function disorder causing systemic inflammation, vasculopathy, and interstitial lung disease. JAK inhibitors are first-line therapy. We report a SAVI patient on prolonged JAK inhibitors who developed opportunistic infections, including fatal Aspergillus endocarditis, underscoring therapeutic challenges.
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