Update in Hyperferritinemic Syndromes: Recognition and Management - A Scoping Review

Abstract

Elevated serum ferritin is a marker of macrophage activation and is associated with increased mortality. The hyperferritinemic syndromes which include hemophagocytic lymphohistiocytosis (HLH) and macrophage activation syndrome (MAS), catastrophic antiphospholipid syndrome (CAPS), septic shock, adult-onset Still’s Disease (AOSD), and multi-inflammatory syndrome related to COVID-19 (MIS-C/A) are characterized by intense inflammation and its sequalae. Prompt recognition and management of these heterogenous disorders is required to improve patient outcomes. We perform a scoping review of the existing literature on the key features of these rare syndromes.