Evaluation of macrophage activation syndrome associated with systemic juvenile idiopathic arthritis: single center experience over a one-year period

Aim: This study aimed to evaluate the demographic, clinical, laboratory properties of patients with macrophage activation syndrome and treatment outcomes. Material and Methods: The data of the patients who were diagnosed with macrophage activation syndrome secondary to systemic juvenile idiopathic arthritis between June 2013-May 2014 were evaluated by screening patient records. Results: Ten patients with macrophage activation syndrome were followed up in one year. The mean age at the time of diagnosis was found to be 7.6 +/- 4.5 years. The most common clinical finding at presentation (80%) was increased body temperature. Hepatosplenomegaly was found in half of the patients. The most common hematological finding (90%) was anemia. The mean erythrocyte sedimentation rate was found to be 71.8 +/- 36.2 mm/h, whereas it was measured to be lower (31.2 +/- 25.2 mm/h) at the time of the diagnosis of macrophage activation syndrome. Increased ferritin level was found in all of our patients (the mean ferritin level was found to be 23 957 +/- 15 525 ng/mL). Hypertriglyceridemia was found in nine patients (90%). The mean triglyceride level was found to be 397 +/- 332 mg/dL. Systemic steroid treatment was administered to all patients. Cyclosporine A was given to eight patients (80%), canakinumab was given to four patients (40%) and anakinra was given to five patients (50%). Plasmapheresis was performed in two patients. Improvement was found in all patients except for one patient. The patient in whom no improvement was observed showed a chronic course. Conclusions: The diagnosis of macrophage activation syndrome should be considered in presence of sudden disturbance in general condition, resistant high fever and systemic inflammation findings in children with active rheumatic disease. Complete recovery can be provided with early and efficient treatment in macrophage activation syndrome which develops secondary to systemic juvenil idiopathic arthritis.