Macrophage Activation Syndrome Complicating Atypical Systemic Onset Juvenile Idiopathic Arthritis in a 4 Years Old Child

4 years old boy presented with prolonged fever for 12 weeks not responding to treatment with antibiotic and antipyretics along with pain, swelling and limitation of movements affecting both ankles, right knee and cervical joints. Erythematous skin rash was there. He had faint systolic murmur at LPSA. Abdominal examination revealed liver palpable 2 cm below costal margin. After one week of hospital admission the patient started to show deterioration in his general condition became more toxic, continuous fever and lethargy. Macular rash started to appear on the face. He developed anemia and thrombocytopenia. Bone marrow aspiration was suggestive of hemophagocytic disorder. Ferritin level was 45651 ng/ml, fibrinogen level 100 (low) triglycerides was high LDH >2000 and ESR became low, liver enzymes were shooting S bilirubin rising and patient had more deterioration in the conscious level. Still he had normal blood pressure good peripheral perfusion no signs of shock. He was diagnosed as potentially fatal macrophage activation syndrome. Received date: 12/12/2016 Accepted date: 12/01/2017 Published date: 19/01/2017