Pathogenesis and Treatment of Refractory Disease Courses in Systemic Juvenile Idiopathic Arthritis: Refractory Arthritis, Recurrent Macrophage Activation Syndrome and Chronic Lung Disease.

Systemic juvenile idiopathic arthritis is a distinct and heterogeneous disease presently classified under the umbrella of juvenile idiopathic arthritis, with some patients following a monophasic remitting course, whereas others have persistent disease with chronic organ- and life-threatening complications. Although biologic therapies have revolutionized treatment, recent follow-up studies report significant numbers of children with persistently active disease on long term follow-up. This review focuses on refractory disease courses, specifically refractory arthritis, systemic juvenile idiopathic arthritis with recurrent, or longstanding signs of macrophage activation syndrome, and systemic juvenile idiopathic arthritis associated with suspected, probable, or definite lung disease.