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Immune cartography of macrophage activation syndrome in the COVID-19 era.
McGonagle, Dennis; Ramanan, Athimalaipet V; Bridgewood, Charlie.
  • McGonagle D; Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, UK. d.g.mcgonagle@leeds.ac.uk.
  • Ramanan AV; National Institute for Health Research (NIHR), Leeds Biomedical Research Centre (BRC), Leeds Teaching Hospitals, Leeds, UK. d.g.mcgonagle@leeds.ac.uk.
  • Bridgewood C; University Hospitals Bristol NHS Foundation Trust & Translational Health Sciences, University of Bristol, Bristol, UK.
Nat Rev Rheumatol ; 17(3): 145-157, 2021 03.
Article in English | MEDLINE | ID: covidwho-1065891
ABSTRACT
A hyperinflammatory 'cytokine storm' state termed macrophage activation syndrome (MAS), culminating from a complex interplay of genetics, immunodeficiency, infectious triggers and dominant innate immune effector responses, can develop across disparate entities including systemic juvenile idiopathic arthritis (sJIA) and its counterpart adult-onset Still disease (AOSD), connective tissue diseases, sepsis, infection, cancers and cancer immunotherapy. Classifying MAS using the immunological disease continuum model, with strict boundaries that define the limits of innate and adaptive immunity, at one boundary is MAS with loss of immune function, as occurs in the 'perforinopathies' and some cases of sJIA-AOSD. Conversely, at the other boundary, immune hypersensitivity with gain of immune function in MHC class II-associated sJIA-AOSD and with chimeric antigen receptor (CAR) T cell therapy also triggers MAS. This provides a benchmark for evaluating severe inflammation in some patients with COVID-19 pneumonia, which cripples primary type I interferon immunity and usually culminates in a lung-centric 'second wave' cytokine-driven alveolitis with associated immunothrombosis; this phenomenon is generally distinct from MAS but can share features with the proposed 'loss of immune function' MAS variant. This loss and gain of function MAS model offers immune cartography for a novel mechanistic classification of MAS with therapeutic implications.
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Full text: Available Collection: International databases Database: MEDLINE Main subject: Cytokines / Macrophage Activation Syndrome / Pandemics / SARS-CoV-2 / COVID-19 / Macrophages Type of study: Experimental Studies / Observational study Topics: Variants Limits: Humans Language: English Journal: Nat Rev Rheumatol Journal subject: Rheumatology Year: 2021 Document Type: Article Affiliation country: S41584-020-00571-1

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Full text: Available Collection: International databases Database: MEDLINE Main subject: Cytokines / Macrophage Activation Syndrome / Pandemics / SARS-CoV-2 / COVID-19 / Macrophages Type of study: Experimental Studies / Observational study Topics: Variants Limits: Humans Language: English Journal: Nat Rev Rheumatol Journal subject: Rheumatology Year: 2021 Document Type: Article Affiliation country: S41584-020-00571-1