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

Abstract

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. (Turk Pediatri Ars 2015; 50: 206-10)

Idiopathic Pulmonary Hemosiderosis in a Child with Recurrent Macrophage Activation Syndrome Secondary to Systemic Juvenile Idiopathic Arthritis

“…An investigation of MAS patients secondary to SJIA that were being followed up at our department during last year showed a mean ferritin level of 23957 ± 15525 ng/mL (minimal: 3000 ng/mL, maximal: 46.130 ng/mL). None of those patients developed pulmonary hemosiderosis [ 1 ]. The serum ferritin levels could be elevated or it could be in normal limits in IPH.…”

“…Systemic juvenile idiopathic arthritis (SJIA) is a rare disease characterized by prolonged and intermittent fever, arthritis, and evanescent rash. Other features in a minority of patients are hepatosplenomegaly, lymphadenopathy, and serositis [ 1 ]. Macrophage activation syndrome (MAS) is the most important and destructive complication of the disease.…”

Juvenile Idiopathic Arthritis

“…The ESR drops sharply in association with hypofibrinogenaemia. Typically, liver enzymes, lactate dehydrogenase (LDH), triglycerides and ferritin levels are elevated, sometimes with extreme hyperferritinaemia, hypoalbuminaemia and hyponatraemia ( 21 ). A European League Against Rheumatism/American College of Rheumatology/Paediatric Rheumatology International Trials Organisation collaborative initiative recently proposed criteria for MAS complicating systemic JIA: febrile patients with confirmed or suspected sJIA with ferritin >684 ng/mL and any two of the following - platelet count ≤181x109/L, aspartate aminotransferase >48 units/L, triglycerides >156 mg/dL, fibrinogen ≤360 mg/dL.…”

Systemic Form of Juvenile Idiopathic Arthritis: Epidemiological, Clinical, Paraclinical and Therapeutic Aspects of 13 Cases in Abidjan

“…The vital prognosis is singularly engaged in the case of macrophage activation syndrome (MAS), which represent 10% of the systemic forms. It is the most important complication of SF-JIA because it can be fatal [40] [41]. In our study, the only case of MAS occurred in a male child and was fatal.…”