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Emapalumab in patients with macrophage activation syndrome

Presented By
Dr Fabrizio De Benedetti, Ospedale Pediatrico Bambino Gesù, Italy
EULAR 2019
Emapalumab, an interferon gamma (IFNγ)-blocking monoclonal antibody, has shown efficacy and safety in patients with macrophage activation syndrome (MAS) complicating systemic juvenile idiopathic arthritis (SJIA) MAS is a rare, systemic life-threatening disorder characterised by uncontrolled hyperinflammation which may develop on a background of rheumatic diseases such as SJIA. It is classified as a secondary form of hemophagocytic lymphohistiocytosis (HLH) and is caused by excessive activation and expansion of T cells and macrophages. In recent years, evidence has shown uncontrolled overproduction of IFNγ as a major driver of hyperinflammation and hypercytokinaemia in diseases such as MAS and HLH. Emapalumab is a monoclonal antibody that binds to and neutralises interferon gamma (IFNγ). Dr Fabrizio De Benedetti et al. (Ospedale Pediatrico Bambino Gesù, Italy) initiated a pilot study which aimed to assess the pharmacokinetics, efficacy, and safety of...

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