From: Proceedings from the 2nd Next Gen Therapies for Systemic Juvenile Idiopathic Arthritis and Macrophage Activation Syndrome symposium held on October 3-4, 2019
Failure to respond to IL-1 AND IL-6 blocking biologics (failure = inability to resolve arthritis, systemic symptoms, or liver dysfunction, or being steroid dependent)
OR
≥2 episodes of MAS in a 2 year period
Development of SJIA-LD