Clinical Quiz: Systemic JIA Treatment Recommendations from 2021 ACR JIA Guidelines

Systemic juvenile idiopathic arthritis (JIA) is considered by some rheumatologists to be an autoinflammatory disorder, with patients experiencing rash, fever, and visceral involvement in addition to the stiff, swollen, and painful joints associated with arthritis. Up to 40% of patients with systemic JIA are also diagnosed with macrophage activation syndrome (MAS), a life-threatening complication that presents with elevated liver enzyme levels, low fibrinogen levels, high triglyceride levels, high ferritin levels, cytopenia, and fevers. Careful monitoring and effective treatment strategies are critical, as this syndrome can occur at any point during the disease course.

In the third installment in a series of clinical quizzes based on the American College of Rheumatology’s 2021 Guideline for the Treatment of Juvenile Idiopathic Arthritis, we focus on treatment recommendations for pediatric patients with systemic JIA, both with and without MAS.