Macrophage activation syndrome due to systemic onset juvenile idiopathic arthritis in a case with liver transplantation

Methods A 9-year-old girl (with a liver transplantation because of biliar hypoplasia from her father at the age of five years) presented with fever, rash and oligoarthiritis. An urgent tube thoracostomy was performed because of severe pleural effusion. She was transferred to the intensive care unit with the diagnosis of sepsis. On admission, the patient’s white blood cells (WBC) 31600/mm, platelets (plt) 40600/mm, C reactive protein (CRP) 264 mg/dl and liver enzymes was normal. Because of persistent fever and pericardial effusion, bone marrow aspiration was performed and revealed hemophagocytosis. Intravenous methylprednisolone (IV) was administered at a dose of 30 mg/kg/day for three days. Fever was reported to subside and her treatment switched to oral methylprednisolone at a dose of 1.5 mg/kg/day. Maculopapular rash and pericardial effusion persisted. At that time the patient’s hemoglobin (Hb) was 9.2 g/dL, WBC 40900/ mm, plt 151000/mm, erythrocyte sedimentation rate (ESR) 8 mm/h, CRP 8.26 mg/dl, fibrinogen 3.7 g/l, alanine aminotransferase (ALT) 93 U/l, aspartate aminotransferase (AST) 69 U/l. After four days, resistant high fever relapsed and dexamethasone, etoposide and IVIG added to her treatment. The dose of tacrolimus given for liver transplantation was increased to 3 mg/day.