Systemic Juvenile Idiopathic Arthritis, Macrophage Activation Syndrome and Cardiopulmonary Involvement in Rheumatic Diseases: A Rheumatologist’s Must-Knows

Systemic juvenile idiopathic arthritis (sJIA) is the most severe variant of JIA that can be characterized by a life-threatening course. sJIA manifests with fever, arthralgia/arthritis, hepatosplenomegaly, leukocytosis and systemic inflammation markers. Macrophage activation syndrome (MAS) is the most severe complication of this disease. This Article represents up-to-date information on pathogeneses of sJIA and MAS, describes approaches to diagnosis, treatment of both uncomplicated and complicated forms of sJIA and refractory variants of the MAS course. Current data regarding the monitoring of sJIA therapy effectiveness and safety is presented as well. Predictors for the most recently discovered, i.e. relatively new “target organs” in patients with sJIA and cardiopulmonary disorders in particular are given.