Macrophage Activation Syndrome in Children with Systemic-Onset Juvenile Chronic Arthritis

Abstract

Macrophage activation syndrome (MAS) is a life-threatening complication in children with rheumatic diseases, particularly systemic-onset juvenile chronic arthritis (SOJCA). Because of the potential fatality of this condition, prompt recognition and immediate therapeutic intervention are important. This study assessed the clinical features of nine MAS events in five children with SOJCA. Nonremitting fever and decreased platelet and white blood cell counts led to a diagnosis of MAS. The urinary β2-microglobulin (β2MG) level was a sensitive indicator of MAS. Serum levels of β2MG and soluble interleukin-2 receptor were also elevated. These biologic markers reflecting hyperactivated cellular immunity are useful indicators of MAS. Four children treated with cyclosporin A (CSP) achieved rapid and complete recovery, but one patient without CSP died due to rapidly progressive respiratory failure. All children treated with CSP responded quickly, and fever abated within 36 h of initiation of treatment. CSP should be added to first-line therapy of MAS.

Macrophage Activation Syndrome in Patients with Systemic Juvenile Idiopathic Arthritis under Treatment with Tocilizumab

“…Cytopenia and hemophagocytosis, progressive apoptosis/necrosis 15,16,20 , elevated cytokine-induced proteins such as ferritin 34 and β 2 -MG 35 , coagulation/fibrinolytic abnormalities 36,37 , and massive endothelial cell damage 38 are the hallmarks of MAS. Excessive IL-1β, TNF-α, IL-6, and IFN-g are likely to contribute to early persistent fevers 39 , production of cytokine-induced proteins 34,35 , bone marrow overactivation 20-29 years. Excessive IL-1β, TNF-α, IL-6, and IFN-g are likely to contribute to early persistent fevers 39 , production of cytokine-induced proteins 34,35 , bone marrow overactivation 20-29 years.…”

The Use of Cyclosporine A in Rheumatology: a 2016 Comprehensive Review

“…CyA has been proven to result in clinical improvement in most patients, being particularly effective in the management of systemic symptoms [180][181][182][183][184][185][186][187]. Importantly, CyA has been reported lifesaving in serious cases of steroid resistant MAS [180].…”

Relapsing macrophage activating syndrome in a 15-year-old girl with Still's disease: a case report

“…The diagnosis of MAS in a patient known to have a rheumatic disease must be suspected when the patient shows signs of systemic derangement, fever, hepatosplenomegaly, bleeding tendency, leukopenia, thrombocytopenia, increase in liver enzyme values and coagulations disturbance [12]. Hypofibrinogenemia is one of the most important clues for the diagnosis of MAS since patients usually have high fibrinogen levels due to their underlying inflammatory disease.…”