Background Macrophage activation syndrome (MAS) is a serious, potentially fatal complication of childhood systemic inflammatory disorders, and it is most frequent in Systemic Juvenile Idiopthic Arthritis, for instance, it is increasingly reported in other pediatric rheumatic diseases as lupus erythematosus and Kawasaki disease.
Objectives To describe clinical case report of a 16 year old girl with Juvenil Systemic Lupus Erythematosus and Macrophage Activation Syndrome refractory.
Methods A 16-year-old woman with recent diagnosis of Systemic Lupus Erythematosus in November 2015. At admission with continuos fever lasting 2 months, with initial laboratory studies with Triglycerides 395 mg/dl, Ferritin 3.300 ug/l, soluble receptor IL-2 2.838 U/ml, fibrinogen 166 mg/dl, Hemophagocytos in bone marrow, presence of persistent cytopenias. Initial management with methylprednisolone 30 mg/kg/day (3 days) without clinical response was initiated. Management is added to Cyclosporin A (10 mg/kg/day), reporting subtherapeutic serum levels despite high doses without clinical response and improvement of laboratory controls. During the hospital stay complete 13 weeks of treatment with Etposide (180 mg/dose), with ferritin levels in 4,180 ug/l, triglycerides 438, Fibrinogen 455 mg/dl, WBC 5,000 ul-1 (3,400 neutrophils, lymphocytes 1,050), platelet count 78 x 103 ml-1, hemoglobin 7.2 g/dl. After three months of treatment, she was given with Etanercept 0.4mg/kg/dose, 2 times a day. Currently in week 3 of treatment with WBC 6,500 ml-1, hemoglobin 9.7 g/dl, platelet count 140 x 103ml-1 and ferritin 4,270 ng/ml. The patient remains disease with costicosteroid, cyclosporine, and etanercept, without adverse events.
Results Patient treated with Etanercept during 5 weeks, presenting clinical response for MAS.
Conclusions A general therapeutic protocol for MAS is not available: first line treatment is usually represented by parenteral administration of high dose corticosteroids. Mild forms are reported to respond to steroids alone in association with supportive medicaments. Steroid-resistant cases or the most severe forms of MAS require the addition of cyclosporine A, other therapeutic regimens have been studied such as high-dose intravenous immunoglobulins, antithymocyte globulins, etanercept, etoposide and plasmapheresis
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Disclosure of Interest None declared