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Janus kinase inhibition for autoinflammation in DNASE2 deficiency

Hong, Y; Capitani, M; Murphy, C; Pandey, S; Cavounidis, A; Takeshita, H; Nanthapisal, S; ... Brogan, P; + view all (2019) Janus kinase inhibition for autoinflammation in DNASE2 deficiency. Journal of Allergy and Clinical Immunology 10.1016/j.jaci.2019.11.020. (In press). Green open access

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Abstract

We characterise the immunopathogenesis of autoinflammation, HLH, and early onset intestinal inflammation caused by homozygous mutation in DNASE2, and provide the first description of favourable therapeutic response to JAK1/JAK2 blockade in this disease.

Type: Article
Title: Janus kinase inhibition for autoinflammation in DNASE2 deficiency
Location: United States
Open access status: An open access version is available from UCL Discovery
DOI: 10.1016/j.jaci.2019.11.020
Publisher version: https://doi.org/10.1016/j.jaci.2019.11.020
Language: English
Additional information: This article is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License, which permits any non-commercial use, sharing, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, and provide a link to the Creative Commons license. You do not have permission under this license to share adapted material derived from this article or parts of it. The images or other third party material in this article are included in the article’s Creative Commons license, unless indicated otherwise in a credit line to the material. If material is not included in the article’s Creative Commons license and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this license, visit http://creativecommons.org/licenses/by-nc-nd/4.0/.
Keywords: DNASE2 deficiency; Type I Interferon; autoinflammation; JAK inhibition; baricitinib; haemophagocytic lymphohistiocytosis; early-onset intestinal inflammation.
UCL classification: UCL
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Div of Medicine
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Div of Medicine > Inflammation
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > UCL GOS Institute of Child Health
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > UCL GOS Institute of Child Health > Infection, Immunity and Inflammation Dept
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > UCL GOS Institute of Child Health > Population, Policy and Practice Dept
URI: https://discovery.ucl.ac.uk/id/eprint/10087078
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