Immune cartography of macrophage activation syndrome in the COVID-19 era.
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.
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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