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Criteria for Cytokine Storm Syndromes

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Cytokine Storm Syndrome

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Abstract

In the past two decades, there has been a great deal of work aimed to devise diagnostic guidelines, classification criteria, and diagnostic scores for cytokine storm syndromes. The most notable effort has been the large-scale multinational study that led to the development of the 2016 classification criteria for macrophage activation syndrome (MAS) complicating systemic juvenile idiopathic arthritis (JIA). In the context of this project, the laboratory tests whose change over time is most valuable for early diagnosis of MAS in systemic JIA were identified. Other tools are the MH score to distinguish MAS from primary hemophagocytic lymphohistiocytosis, the diagnostic guidelines for MAS in childhood-onset systemic lupus erythematosus, the HScore for reactive hemophagocytic syndromes, and the MS score for early detection of MAS in systemic JIA. Future studies should scrutinize the validity of proposed criteria in different patient populations evaluated prospectively. There is also the specific need to assess the diagnostic performance of criteria in patients with systemic JIA treated with biologics.

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Authors and Affiliations

  1. Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan, Italy

    Francesca Minoia

  2. IRCCS Istituto Giannina Gaslini, Genoa, Italy

    Sergio Davì & Angelo Ravelli

  3. Università degli Studi di Genova, Genoa, Italy

    Alessandra Alongi & Angelo Ravelli

Authors
  1. Francesca Minoia
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  2. Sergio Davì
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  3. Alessandra Alongi
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  4. Angelo Ravelli
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Correspondence to Angelo Ravelli .

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Editors and Affiliations

  1. UAB School of Medicine, University of Alabama at Birmingham, Birmingham, AL, USA

    Randy Q. Cron

  2. Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA

    Edward M. Behrens

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Minoia, F., Davì, S., Alongi, A., Ravelli, A. (2019). Criteria for Cytokine Storm Syndromes. In: Cron, R., Behrens, E. (eds) Cytokine Storm Syndrome. Springer, Cham. https://doi.org/10.1007/978-3-030-22094-5_5

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