The Role of Interferon-γ in Autoimmune Polyendocrine Syndrome Type 1.

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Fältnamn	Indikatorer	Metadata
000		07651naa a2201477 4500
001		oai:gup.ub.gu.se/338274
003		SwePub
008		240613s2024 \| \|\|\|\|\|\|\|\|\|\|\|000 \|\|eng\|
024	7	a https://gup.ub.gu.se/publication/3382742 URI
024	7	a https://doi.org/10.1056/NEJMoa23126652 DOI
040		a (SwePub)gu
041		a eng
042		9 SwePub
072	7	a ref2 swepub-contenttype
072	7	a art2 swepub-publicationtype
100	1	a Oikonomou, Vasileios4 aut
245	1 0	a The Role of Interferon-γ in Autoimmune Polyendocrine Syndrome Type 1.
264	1	c 2024
520		a Autoimmune polyendocrine syndrome type 1 (APS-1) is a life-threatening, autosomal recessive syndrome caused by autoimmune regulator (AIRE) deficiency. In APS-1, self-reactive T cells escape thymic negative selection, infiltrate organs, and drive autoimmune injury. The effector mechanisms governing T-cell-mediated damage in APS-1 remain poorly understood.We examined whether APS-1 could be classified as a disease mediated by interferon-γ. We first assessed patients with APS-1 who were participating in a prospective natural history study and evaluated mRNA and protein expression in blood and tissues. We then examined the pathogenic role of interferon-γ using Aire-/-Ifng-/- mice and Aire-/- mice treated with the Janus kinase (JAK) inhibitor ruxolitinib. On the basis of our findings, we used ruxolitinib to treat five patients with APS-1 and assessed clinical, immunologic, histologic, transcriptional, and autoantibody responses.Patients with APS-1 had enhanced interferon-γ responses in blood and in all examined autoimmunity-affected tissues. Aire-/- mice had selectively increased interferon-γ production by T cells and enhanced interferon-γ, phosphorylated signal transducer and activator of transcription 1 (pSTAT1), and CXCL9 signals in multiple organs. Ifng ablation or ruxolitinib-induced JAK-STAT blockade in Aire-/- mice normalized interferon-γ responses and averted T-cell infiltration and damage in organs. Ruxolitinib treatment of five patients with APS-1 led to decreased levels of T-cell-derived interferon-γ, normalized interferon-γ and CXCL9 levels, and remission of alopecia, oral candidiasis, nail dystrophy, gastritis, enteritis, arthritis, Sjögren's-like syndrome, urticaria, and thyroiditis. No serious adverse effects from ruxolitinib were identified in these patients.Our findings indicate that APS-1, which is caused by AIRE deficiency, is characterized by excessive, multiorgan interferon-γ-mediated responses. JAK inhibition with ruxolitinib in five patients showed promising results. (Funded by the National Institute of Allergy and Infectious Diseases and others.).
650	7	a MEDICIN OCH HÄLSOVETENSKAPx Klinisk medicinx Pediatrik0 (SwePub)302212 hsv//swe
650	7	a MEDICAL AND HEALTH SCIENCESx Clinical Medicinex Pediatrics0 (SwePub)302212 hsv//eng
650	7	a MEDICIN OCH HÄLSOVETENSKAPx Medicinska och farmaceutiska grundvetenskaperx Immunologi inom det medicinska området0 (SwePub)301102 hsv//swe
650	7	a MEDICAL AND HEALTH SCIENCESx Basic Medicinex Immunology in the medical area0 (SwePub)301102 hsv//eng
653		a Polyendocrinopathies
653		a Autoimmune
653		a genetics
653		a drug therapy
653		a immunology
653		a Interferon-gamma
653		a metabolism
653		a genetics
653		a Humans
653		a Animals
653		a Nitriles
653		a therapeutic use
653		a Mice
653		a Pyrazoles
653		a therapeutic use
653		a pharmacology
653		a Pyrimidines
653		a therapeutic use
653		a Transcription Factors
653		a genetics
653		a AIRE Protein
653		a Female
653		a Male
653		a Mice
653		a Knockout
653		a Adult
653		a Chemokine CXCL9
653		a genetics
653		a Autoantibodies
653		a blood
653		a T-Lymphocytes
653		a immunology
653		a Janus Kinase Inhibitors
653		a therapeutic use
700	1	a Smith, Grace4 aut
700	1	a Constantine, Gregory M4 aut
700	1	a Schmitt, Monica M4 aut
700	1	a Ferré, Elise M N4 aut
700	1	a Alejo, Julie C4 aut
700	1	a Riley, Deanna4 aut
700	1	a Kumar, Dhaneshwar4 aut
700	1	a Dos Santos Dias, Lucas4 aut
700	1	a Pechacek, Joseph4 aut
700	1	a Hadjiyannis, Yannis4 aut
700	1	a Webb, Taura4 aut
700	1	a Seifert, Bryce A4 aut
700	1	a Ghosh, Rajarshi4 aut
700	1	a Walkiewicz, Magdalena4 aut
700	1	a Martin, Daniel4 aut
700	1	a Besnard, Marine4 aut
700	1	a Snarr, Brendan D4 aut
700	1	a Deljookorani, Shiva4 aut
700	1	a Lee, Chyi-Chia R4 aut
700	1	a DiMaggio, Tom4 aut
700	1	a Barber, Princess4 aut
700	1	a Rosen, Lindsey B4 aut
700	1	a Cheng, Aristine4 aut
700	1	a Rastegar, Andre4 aut
700	1	a de Jesus, Adriana A4 aut
700	1	a Stoddard, Jennifer4 aut
700	1	a Kuehn, Hye Sun4 aut
700	1	a Break, Timothy J4 aut
700	1	a Kong, Heidi H4 aut
700	1	a Castelo-Soccio, Leslie4 aut
700	1	a Colton, Ben4 aut
700	1	a Warner, Blake M4 aut
700	1	a Kleiner, David E4 aut
700	1	a Quezado, Martha M4 aut
700	1	a Davis, Jeremy L4 aut
700	1	a Fennelly, Kevin P4 aut
700	1	a Olivier, Kenneth N4 aut
700	1	a Rosenzweig, Sergio D4 aut
700	1	a Suffredini, Anthony F4 aut
700	1	a Anderson, Mark S4 aut
700	1	a Swidergall, Marc4 aut
700	1	a Guillonneau, Carole4 aut
700	1	a Notarangelo, Luigi D4 aut
700	1	a Goldbach-Mansky, Raphaela4 aut
700	1	a Neth, Olaf4 aut
700	1	a Monserrat-Garcia, Maria Teresa4 aut
700	1	a Valverde-Fernandez, Justo4 aut
700	1	a Lucena, Jose Manuel4 aut
700	1	a Gomez-Gila, Ana Lucia4 aut
700	1	a Garcia Rojas, Angela4 aut
700	1	a Seppänen, Mikko R J4 aut
700	1	a Lohi, Jouko4 aut
700	1	a Hero, Matti4 aut
700	1	a Laakso, Saila4 aut
700	1	a Klemetti, Paula4 aut
700	1	a Lundberg, Vanjau Gothenburg University,Göteborgs universitet,Institutionen för kliniska vetenskaper, Avdelningen för pediatrik,Institutionen för medicin, avdelningen för reumatologi och inflammationsforskning,Institute of Clinical Sciences, Department of Pediatrics,Institute of Medicine, Department of Rheumatology and Inflammation Research4 aut0 (Swepub:gu)xlunva
700	1	a Ekwall, Olov,d 1968u Gothenburg University,Göteborgs universitet,Institutionen för medicin, avdelningen för reumatologi och inflammationsforskning,Institutionen för kliniska vetenskaper, Avdelningen för pediatrik,Institute of Medicine, Department of Rheumatology and Inflammation Research,Institute of Clinical Sciences, Department of Pediatrics4 aut0 (Swepub:gu)xekwol
700	1	a Olbrich, Peter4 aut
700	1	a Winer, Karen K4 aut
700	1	a Afzali, Behdad4 aut
700	1	a Moutsopoulos, Niki M4 aut
700	1	a Holland, Steven M4 aut
700	1	a Heller, Theo4 aut
700	1	a Pittaluga, Stefania4 aut
700	1	a Lionakis, Michail S4 aut
710	2	a Göteborgs universitetb Institutionen för kliniska vetenskaper, Avdelningen för pediatrik4 org
773	0	t The New England journal of medicineg 390:20, s. 1873-1884q 390:20<1873-1884x 1533-4406
856	4 8	u https://gup.ub.gu.se/publication/338274
856	4 8	u https://doi.org/10.1056/NEJMoa2312665

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Av författaren/redakt...: Oikonomou, Vasil ...; Smith, Grace; Constantine, Gre ...; Schmitt, Monica ...; Ferré, Elise M N; Alejo, Julie C; visa fler...; Riley, Deanna; Kumar, Dhaneshwa ...; Dos Santos Dias, ...; Pechacek, Joseph; Hadjiyannis, Yan ...; Webb, Taura; Seifert, Bryce A; Ghosh, Rajarshi; Walkiewicz, Magd ...; Martin, Daniel; Besnard, Marine; Snarr, Brendan D; Deljookorani, Sh ...; Lee, Chyi-Chia R; DiMaggio, Tom; Barber, Princess; Rosen, Lindsey B; Cheng, Aristine; Rastegar, Andre; de Jesus, Adrian ...; Stoddard, Jennif ...; Kuehn, Hye Sun; Break, Timothy J; Kong, Heidi H; Castelo-Soccio, ...; Colton, Ben; Warner, Blake M; Kleiner, David E; Quezado, Martha ...; Davis, Jeremy L; Fennelly, Kevin ...; Olivier, Kenneth ...; Rosenzweig, Serg ...; Suffredini, Anth ...; Anderson, Mark S; Swidergall, Marc; Guillonneau, Car ...; Notarangelo, Lui ...; Goldbach-Mansky, ...; Neth, Olaf; Monserrat-Garcia ...; Valverde-Fernand ...; Lucena, Jose Man ...; Gomez-Gila, Ana ...; Garcia Rojas, An ...; Seppänen, Mikko ...; Lohi, Jouko; Hero, Matti; Laakso, Saila; Klemetti, Paula; Lundberg, Vanja; Ekwall, Olov, 19 ...; Olbrich, Peter; Winer, Karen K; Afzali, Behdad; Moutsopoulos, Ni ...; Holland, Steven ...; Heller, Theo; Pittaluga, Stefa ...; Lionakis, Michai ...; visa färre...

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