Rosain, Jérémie (56023788900)Jérémie (56023788900)RosainVoyer, Tom Le (58681065900)Tom Le (58681065900)VoyerLiu, Xian (58671686500)Xian (58671686500)LiuGervais, Adrian (57222020896)Adrian (57222020896)GervaisPolivka, Laura (55348398600)Laura (55348398600)PolivkaCederholm, Axel (58686435400)Axel (58686435400)CederholmBerteloot, Laureline (16174402700)Laureline (16174402700)BertelootParent, Audrey V. (23486336100)Audrey V. (23486336100)ParentPescatore, Alessandra (22954304800)Alessandra (22954304800)PescatoreSpinosa, Ezia (57416511900)Ezia (57416511900)SpinosaMinic, Snezana (35409907200)Snezana (35409907200)MinicKiszewski, Ana Elisa (36829537900)Ana Elisa (36829537900)KiszewskiTsumura, Miyuki (20435156200)Miyuki (20435156200)TsumuraThibault, Chloé (57393783200)Chloé (57393783200)ThibaultAzcoiti, Maria Esnaola (56543529300)Maria Esnaola (56543529300)AzcoitiMartinovic, Jelena (7006812932)Jelena (7006812932)MartinovicPhilippot, Quentin (56155126800)Quentin (56155126800)PhilippotKhan, Taushif (56519165100)Taushif (56519165100)KhanMarchal, Astrid (57876715800)Astrid (57876715800)MarchalMuylder, Bénédicte Charmeteau-De (58044249000)Bénédicte Charmeteau-De (58044249000)MuylderBizien, Lucy (57219616192)Lucy (57219616192)BizienDeswarte, Caroline (15822072600)Caroline (15822072600)DeswarteHadjem, Lillia (59423388900)Lillia (59423388900)HadjemFauvarque, Marie-Odile (6603031105)Marie-Odile (6603031105)FauvarqueDorgham, Karim (6508287019)Karim (6508287019)DorghamEriksson, Daniel (57095712200)Daniel (57095712200)ErikssonFalcone, Emilia Liana (56850172900)Emilia Liana (56850172900)FalconePuel, Mathilde (57914756700)Mathilde (57914756700)PuelÜnal, Sinem (59423389000)Sinem (59423389000)ÜnalGeraldo, Amyrath (57219655260)Amyrath (57219655260)GeraldoFloc’h, Corentin Le (58919052000)Corentin Le (58919052000)Floc’hLi, Hailun (58681201300)Hailun (58681201300)LiRheault, Sylvie (57211042601)Sylvie (57211042601)RheaultMuti, Christine (6602414824)Christine (6602414824)MutiBobrie-Moyrand, Claire (57221800362)Claire (57221800362)Bobrie-MoyrandWelfringer-Morin, Anne (57195278263)Anne (57195278263)Welfringer-MorinFuleihan, Ramsay L. (7003440477)Ramsay L. (7003440477)FuleihanLévy, Romain (56946108200)Romain (56946108200)LévyRoelens, Marie (57190179406)Marie (57190179406)RoelensGao, Liwei (59317871200)Liwei (59317871200)GaoMaterna, Marie (57215894832)Marie (57215894832)MaternaPellegrini, Silvia (56533440400)Silvia (56533440400)PellegriniPiemonti, Lorenzo (6603924799)Lorenzo (6603924799)PiemontiCatherinot, Emilie (57193275770)Emilie (57193275770)CatherinotGoffard, Jean-Christophe (6506658107)Jean-Christophe (6506658107)GoffardFekkar, Arnaud (24365959200)Arnaud (24365959200)FekkarSacko-Sow, Aissata (59423177600)Aissata (59423177600)Sacko-SowSoudée, Camille (57803366200)Camille (57803366200)SoudéeBoucherit, Soraya (35313297800)Soraya (35313297800)BoucheritNeehus, Anna-Lena (56798534900)Anna-Lena (56798534900)NeehusHas, Cristina (55874488300)Cristina (55874488300)HasHübner, Stefanie (57226530504)Stefanie (57226530504)HübnerBlanchard-Rohner, Géraldine (24070082100)Géraldine (24070082100)Blanchard-RohnerAmador-Borrero, Blanca (57216634371)Blanca (57216634371)Amador-BorreroUtsumi, Takanori (57393787700)Takanori (57393787700)UtsumiTaniguchi, Maki (57221963011)Maki (57221963011)TaniguchiTani, Hiroo (57193084243)Hiroo (57193084243)TaniIzawa, Kazushi (42761574600)Kazushi (42761574600)IzawaYasumi, Takahiro (6603644396)Takahiro (6603644396)YasumiKanai, Sotaro (57190375813)Sotaro (57190375813)KanaiMigaud, Mélanie (37112565200)Mélanie (37112565200)MigaudAubart, Mélodie (56159372600)Mélodie (56159372600)AubartLambert, Nathalie (56463634100)Nathalie (56463634100)LambertGorochov, Guy (7004408016)Guy (7004408016)GorochovPicard, Capucine (57194509265)Capucine (57194509265)PicardSoudais, Claire (6602121088)Claire (6602121088)SoudaisL’honneur, Anne-Sophie (57193070067)Anne-Sophie (57193070067)L’honneurRozenberg, Flore (7004000569)Flore (7004000569)RozenbergMilner, Joshua D. (8335416800)Joshua D. (8335416800)MilnerZhang, Shen-Ying (36019693200)Shen-Ying (36019693200)ZhangVabres, Pierre (7004015324)Pierre (7004015324)VabresTrpinac, Dusan (6602163849)Dusan (6602163849)TrpinacMarr, Nico (24344670100)Nico (24344670100)MarrBoddaert, Nathalie (57203073518)Nathalie (57203073518)BoddaertDesguerre, Isabelle (7003904896)Isabelle (7003904896)DesguerrePasparakis, Manolis (6701908718)Manolis (6701908718)PasparakisMiller, Corey N. (55812657600)Corey N. (55812657600)MillerPoziomczyk, Cláudia S. (8898247600)Cláudia S. (8898247600)PoziomczykAbel, Laurent (7103216988)Laurent (7103216988)AbelOkada, Satoshi (55256600100)Satoshi (55256600100)OkadaJouanguy, Emmanuelle (6701731774)Emmanuelle (6701731774)JouanguyCheynier, Rémi (6603688690)Rémi (6603688690)CheynierZhang, Qian (57201744949)Qian (57201744949)ZhangCobat, Aurélie (11140506200)Aurélie (11140506200)CobatBéziat, Vivien (25642778900)Vivien (25642778900)BéziatBoisson, Bertrand (23484212500)Bertrand (23484212500)BoissonSteffann, Julie (6603035641)Julie (6603035641)SteffannFusco, Francesca (7006608197)Francesca (7006608197)FuscoUrsini, Matilde Valeria (6603768011)Matilde Valeria (6603768011)UrsiniHadj-Rabia, Smail (6603382575)Smail (6603382575)Hadj-RabiaBodemer, Christine (7006485009)Christine (7006485009)BodemerBustamante, Jacinta (57201082594)Jacinta (57201082594)BustamanteLuche, Hervé (15822336300)Hervé (15822336300)LuchePuel, Anne (6602102891)Anne (6602102891)PuelCourtois, Gilles (7004302174)Gilles (7004302174)CourtoisBastard, Paul (57219618314)Paul (57219618314)BastardLandegren, Nils (56649370900)Nils (56649370900)LandegrenAnderson, Mark S. (55682219200)Mark S. (55682219200)AndersonCasanova, Jean-Laurent (7201863327)Jean-Laurent (7201863327)Casanova2025-06-122025-06-122024https://doi.org/10.1084/jem.20231152https://www.scopus.com/inward/record.uri?eid=2-s2.0-85209889207&doi=10.1084%2fjem.20231152&partnerID=40&md5=6e1ec5e532d0826974757be23db4d2c1https://remedy.med.bg.ac.rs/handle/123456789/823Human inborn errors of thymic T cell tolerance underlie the production of autoantibodies (auto-Abs) neutralizing type I IFNs, which predispose to severe viral diseases. We analyze 131 female patients with X-linked dominant incontinentia pigmenti (IP), heterozygous for loss-of-function (LOF) NEMO variants, from 99 kindreds in 10 countries. Forty-seven of these patients (36%) have auto-Abs neutralizing IFN-α and/or IFN-ω, a proportion 23 times higher than that for age-matched female controls. This proportion remains stable from the age of 6 years onward. On imaging, female patients with IP have a small, abnormally structured thymus. Auto-Abs against type I IFNs confer a predisposition to life-threatening viral diseases. By contrast, patients with IP lacking auto-Abs against type I IFNs are at no particular risk of viral disease. These results suggest that IP accelerates thymic involution, thereby underlying the production of auto-Abs neutralizing type I IFNs in at least a third of female patients with IP, predisposing them to life-threatening viral diseases. © 2024 Rosain et al.