An important complication of a child with juvenile idiopathic arthritis: macrophage activation syndrome

• Published in: BMJ case reports 2013-09, Vol.2013, p.bcr2013200397
• Main Authors: Reddy, Yuvaram N V, Bhatia, Navin, Scott, Julius Xavier, Nagarajan, Priyathersini
• Format: Article
• Language: English
• Subjects: Antibiotics; Arthritis; Arthritis, Juvenile - complications; Asia; Blood; Blood platelets; Bone marrow; Bone Marrow - pathology; Child, Preschool; Cytokines; Dehydrogenases; Female; Fever; Hemoglobin; Humans; Indian Sub-Continent; Inflammatory diseases; Liver; Lupus; Lymphocytes; Macrophage Activation Syndrome - etiology; Macrophage Activation Syndrome - pathology; Malaria; Reminder of Important Clinical Lesson; Tropical diseases; Tuberculosis; Tumor necrosis factor-TNF
• ISSN: 1757-790X; 1757-790X
• DOI: 10.1136/bcr-2013-200397

A 4-year-old girl, a known case of juvenile idiopathic arthritis for 2 years presented to us with high-grade fever and abdominal distension for 2 months. On examination, her temperature was 104°F and she was found to be pale with bilateral cervical lymphadenopathy of up to 3×3 cm in size. Her liver was enlarged with a firm consistency and a span of 12 cm. Her spleen was enlarged up to 3 cm along its long axis. The rest of her systemic examination was normal. Laboratory investigations revealed leucocytosis, anaemia and thrombocytopenia with a mildly elevated erythrocyte sedimentation rate. Serum ferritin was 16 500 ng/dL and lactate dehydrogenase was 2311 U/L. A bone marrow aspirate showed macrophages showing ingested nuclei. She was diagnosed as having macrophage activation syndrome and was initiated on intravenous methylprednisolone 300 mg daily for 3 days and was switched over to oral prednisolone 2 mg/kg/day. She is currently doing well on follow-up.