Al-Hakim, A., Mistry, A. and Savic, S. orcid.org/0000-0001-7910-0554 (2022) Improving Diagnosis and Clinical Management of Acquired Systemic Autoinflammatory Diseases. Journal of Inflammation Research, 15. pp. 5739-5755. ISSN 1178-7031
Abstract
Systemic autoinflammatory diseases (SAID) are conditions caused by dysregulation or disturbance of the innate immune system, with neutrophils and macrophages the main effector cells. Although there are now more than 40 distinct, genetically defined SAIDs, the genetic/molecular diagnosis remains unknown for a significant proportion of patients with the disease onset in adulthood. This review focuses on new developments related to acquired/late onset SAID, including phenocopies of monogenic disorders, Schnitzler’s syndrome, Adult onset Still’s disease, VEXAS syndrome, and autoinflammatory complications associated with myelodysplastic syndrome.
Metadata
Item Type: | Article |
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Authors/Creators: |
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Copyright, Publisher and Additional Information: | © 2022 Al-Hakim et al. This work is published and licensed by Dove Medical Press Limited. This is an open access article under the terms of the Creative Commons Attribution License (CC-BY-NC 3.0), which permits unrestricted use, distribution and reproduction in any medium, provided the original work is properly cited. |
Keywords: | autoinflammation, adult onset Still’s disease, inflammasome, VEXAS, Schnitzler’s syndrome |
Dates: |
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Institution: | The University of Leeds |
Academic Units: | The University of Leeds > Faculty of Medicine and Health (Leeds) > School of Medicine (Leeds) > Institute of Rheumatology & Musculoskeletal Medicine (LIRMM) (Leeds) > Inflammatory Arthritis (Leeds) |
Depositing User: | Symplectic Publications |
Date Deposited: | 15 Jan 2024 13:07 |
Last Modified: | 15 Jan 2024 13:07 |
Published Version: | http://dx.doi.org/10.2147/jir.s343261 |
Status: | Published |
Publisher: | Informa UK Limited |
Identification Number: | 10.2147/jir.s343261 |
Related URLs: | |
Open Archives Initiative ID (OAI ID): | oai:eprints.whiterose.ac.uk:207004 |
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