Table 4.

Therapy approach.

I	Immunoglobulins	1 cycle of 2 g/kg body weight given in 2 days
II	Cortisone	Methylprednisolone 20–30 mg/kg/day (maximum 1 g/day) for 3 days, then 1–2 mg/kg/d until day 7, then linear reduction over 2–3 weeks
III	Acetylsalicylic acid	30–50 mg/kg/day until the patient has no fever for 48 h, then a low dose 3–5 mg/kg/day for 6 weeks
IV	Antibiotics	Piperacillin/tazobactam (preferred in cases of abdominal problems), cefotaxim (along with daptomycin), additional clindamycin in cases of signs of shock
Adjuvant therapy	Gastric protection	PPI 1–2 mg/day until cortisone is reduced
	Thrombosis prophylaxis	Low-molecular heparin or continuous heparin
	Biologic drugs	IL-1 inhibitor (anakinra), or IL-6 inhibitor (tocilizumab), or TNF-α inhibitor (infliximab)