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Systemic Lupus Erythematosus and Cytokine Storm

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Cytokine Storm Syndrome

Abstract

SLE is the prototype of autoimmune diseases and can manifest with a plethora of clinical signs and symptoms associated with a myriad of laboratory abnormalities. An infrequent but potentially lethal complication of SLE is macrophage activation syndrome (MAS). The diagnosis of MAS in SLE can be very challenging due to similarities in presentation of both flares and infections, such as fever, lymphadenopathy, splenomegaly, and cytopenias. These aggravating factors contribute to the increased risk of poor outcome in SLE-associated MAS. Indeed, MAS at the moment remains invariably lethal if untreated and still has high mortality rate with treatment. In this chapter, we discuss several aspects of MAS in the context of SLE. In particular, we discuss the pathogenesis of MAS in SLE, how MAS presents in pediatric versus adult SLE, and, finally, MAS treatment in SLE and future directions.

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Authors and Affiliations

  1. Lewis Katz School of Medicine, Temple University, Philadelphia, PA, USA

    Roberto Caricchio MD

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Correspondence to Roberto Caricchio MD .

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  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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Caricchio, R. (2019). Systemic Lupus Erythematosus and Cytokine Storm. In: Cron, R., Behrens, E. (eds) Cytokine Storm Syndrome. Springer, Cham. https://doi.org/10.1007/978-3-030-22094-5_22

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