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Table 3 Empirical immunomodulatory therapeutics for rheumatic diseases associated with MAS [11, 31,32,33,34]

From: Hemophagocytic lymphohistiocytosis and macrophage activation syndrome: two rare sides of the same devastating coin

Therapeutic agent

Target/action

Dosing

Glucocorticoids [11, 33]

Broad immunosuppression

a- Methylprednisolone IV or PO prednisone 1–2 mg/kg/day

b- Methylprednisolone IV 10–30 mg/kg/day (maximum 1 g/day for 3 follow-up days)

c- Dexamethasone IV/PO 10 mg/m²/day

Anakinra (rhIL-1Ra) [11, 33, 34]

Blocks IL-1 receptor binding

5–10 mg/kg/day IV/SC q6-12 hr

Intravenous immunoglobulin (IVIG) [11, 33]

Multiple targets

1–2 g/kg/day × 2 days IV

  1. IV Intravenous, mg Miligrams, PO Oral use, SC Subcutaneous, rhIL-1Ra Recombinant human IL-1 receptor antagonista, IVIG Intravenous immunoglobulin
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Advances in Rheumatology

ISSN: 2523-3106