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Systemic juvenile idiopathic arthritis (SJIA) is characterized by fevers, rash, and chronic arthritis, and interleukin -1 (IL-1) and IL-6 inhibitors seems to be effective treatments. Pulmonary arterial hypertension (PAH) and macrophage activation syndrome (MAS), which is a unremitting fever, coagulopathy, pancytopenia, and multiple organ dysfunction. These complications can be fatal and may be the result of severe uncontrolled systemic disease activity or influenced by medication exposure.