RT Journal Article
SR Electronic
T1 Hyperferritinemia Wins Again: Defining Macrophage Activation Syndrome in Pediatric Systemic Lupus Erythematosus
JF The Journal of Rheumatology
JO J Rheumatol
FD The Journal of Rheumatology
SP jrheum.210024
DO 10.3899/jrheum.210024
A1 Emily A.  Smitherman
A1 Randy Q.  Cron
YR 2021
UL http://www.jrheum.org/content/early/2021/06/10/jrheum.210024.abstract
AB Macrophage activation syndrome (MAS) is a potentially life-threatening condition of hyperinflammation that can be secondary to an underlying chronic rheumatic condition, commonly systemic juvenile idiopathic arthritis (sJIA) but also childhood-onset systemic lupus erythematosus (cSLE). MAS is characterized by excessive activation of T lymphocytes and macrophages that lead to overproduction of cytokines and results in cytopenia, liver dysfunction, and coagulopathy1.