TY - JOUR T1 - Higher-dose Anakinra Is Effective in a Case of Medically Refractory Macrophage Activation Syndrome JF - The Journal of Rheumatology JO - J Rheumatol SP - 743 LP - 744 DO - 10.3899/jrheum.121098 VL - 40 IS - 5 AU - PHILIP J. KAHN AU - RANDY Q. CRON Y1 - 2013/05/01 UR - http://www.jrheum.org/content/40/5/743.abstract N2 - To the Editor:An 11-year-old girl was diagnosed with systemic juvenile idiopathic arthritis (sJIA) after presenting with prolonged fever, lymphadenopathy, arthritis, classic sJIA rash, and hyperferritinemia. Complicating macrophage activation syndrome (MAS) was diagnosed, noting hemophagocytosis on bone marrow biopsy for further evaluation of mental status changes. Despite high-dose glucocorticoids and cyclosporine, she remained febrile until the addition of daily subcutaneous interleukin 1 (IL-1) receptor antagonist anakinra (100 mg).She later developed a methicillin-resistant Staphylococcus aureus abscess, and anakinra was withheld. After several days of intravenous antibiotics, she was discharged while taking oral antibiotics, prednisone, and cyclosporine. After anakinra had been withheld for 14 days, she was rehospitalized, this time for a flare of her sJIA, with fever, hypotension, and doubling of her ferritin level (9000 ng/ml). Daily anakinra was resumed, and she received solumedrol pulse therapy and intravenous cyclosporine for persistent fever and hyperferritinemia (peaking at 100,000 ng/ml). She was eventually discharged while taking cyclosporine, prednisone, and anakinra. She remained clinically quiescent for several weeks without fever and her serum ferritin decreased (300 ng/ml).However, because she developed significant medication toxicity (weight gain, hypertension, hirsuitism, and paresthesias), her corticosteroid dose was tapered. She was soon readmitted with anorexia, malaise, and fever. Her medications on admission included … Address correspondence to Dr. R.Q. Cron, Children’s Hospital of Alabama, 1600 7th Avenue South, CPP 210, Birmingham, AL 35233-1711, USA. E-mail: rcron{at}peds.uab.edu ER -