High GC doses (IV pulsed methylprednisolone dose as above; or dexamethasone 10 mg/m2/daily IV) followed by oral prednisolone (1–2 mg/kg/day), in combination with IVIG or IL-1 inhibition, depending on aetiology and severity, are also recommended as first-line therapy in hemophagocytic lymphohistiocytosis (HLH)/macrophage activation syndrome (MAS) with persistent and severe inflammation and organ dysfunction [18, 19]. Here, IL1B is linked to macrophage activation syndrome.