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IL-1 Trap rilonacept in refractory adult onset Still's disease
  1. Olga Petryna1,
  2. J Jack Cush2,
  3. Petros Efthimiou3
  1. 1Medicine/Rheumatology, Columbia University College of Physicians and Surgeons, New York, USA
  2. 2Department of Rheumatology, Baylor University Medical Center, Baylor Research Institute, Dallas, Texas, USA
  3. 3Division of Rheumatogy, Lincoln Medical and Mental Health Center and Weill Cornell Medical College of Cornell University, New York, USA
  1. Correspondence to Petros Efthimiou, Division of Rheumatology, Lincoln Medical and Mental Health, Bronx, NY 10451, USA; pe53{at}cornell.edu

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Adult onset Still's disease (AOSD) is a seronegative polyarthritis associated with rash and fever.1 The innate immune system has been implicated in its pathogenesis and hypersecretion of interleukin 1 (IL-1) has been associated with increased disease activity.2 We report the first successful use of rilonacept, a long-acting IL-1 Trap, in the management of three patients with refractory AOSD. Rilonacept is a soluble dimeric fusion protein (IL-1 receptor extracellular domain +hIgG1-Fc) administered as a weekly subcutaneous injection (220 mg loading dose and 160 mg maintenance dose).3

Patient 1 was a 41 year-old woman who presented with spiking fevers, sore throat, urticaria, arthritis and splenomegaly accompanied by leucocytosis, elevated erythrocyte sedimentation rate (>100 mm/h), increased lung function tests and hyperferritinaemia (8442 ng/ml). …

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