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Published in: Rheumatology International 1/2020

Open Access 01-01-2020 | Cytokines | Case Based Review

Adult-onset Still’s disease with macrophage activation syndrome diagnosed and treated based on cytokine profiling: a case-based review

Authors: Ken Goda, Tsuneaki Kenzaka, Masahiko Hoshijima, Akihiro Yachie, Hozuka Akita

Published in: Rheumatology International | Issue 1/2020

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Abstract

Adult-onset Still’s disease (AOSD) is a relatively rare systemic inflammatory disorder and is diagnosed using various sets of classification criteria, with the Yamaguchi criteria as the most widely used criteria. Herein, we present the case of a 21-year-old woman admitted with a high fever, lasting for over 1 month, who did not fulfill the Yamaguchi criteria. However, by analyzing the inflammatory cytokine profile, we defined this case as AOSD based on a greatly elevated serum interleukin-18 level. In addition, we predicted the occurrence of macrophage activation syndrome by a characteristic increase in the soluble tumor necrosis factor receptor II level, which allowed a timely intervention for this malicious complication. Therefore, we suggest that cytokine profiling will be useful for the diagnosis and management of AOSD.
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Metadata
Title
Adult-onset Still’s disease with macrophage activation syndrome diagnosed and treated based on cytokine profiling: a case-based review
Authors
Ken Goda
Tsuneaki Kenzaka
Masahiko Hoshijima
Akihiro Yachie
Hozuka Akita
Publication date
01-01-2020
Publisher
Springer Berlin Heidelberg
Keyword
Cytokines
Published in
Rheumatology International / Issue 1/2020
Print ISSN: 0172-8172
Electronic ISSN: 1437-160X
DOI
https://doi.org/10.1007/s00296-019-04446-x

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