Pediatric Rheumatology Online Journal → June 2003 → Oligoarthritis→ Abstract #19

EPSTEIN-BARR VIRUS-ASSOCIATED MACROPHAGE ACTIVATION SYNDROME IN OLIGOARTHRITIS

M. Henrickson,¹ B. J. Bloom.²

¹Division of Rheumatology, Children's Hospital Central California, Madera, CA; ²Pfizer Inc., New London, CT

Macrophage activation syndrome (MAS) comprises a rare, heterogeneous group of disorders, principally typified by fever, hemorrhage, coagulopathy, hepatitis and mental status changes, resulting from systemic histiocytic proliferation. In addition to several disease associations, infections or medications may precipitate MAS. This syndrome may seldom complicate polyarthritis and systemic arthritis, but MAS has not been reported in oligoarthritis. We describe 2 girls with persistent oligoarthritis who developed MAS in association with Epstein-Barr virus (EBV). Both had unremitting high fever, anemia, thrombocytopenia, markedly elevated liver function tests and D-dimer formation; case 2 also had disseminated intravascular coagulation, hypertriglyceridemia, ferritinemia and highly elevated cytokine levels. Case 1. A 4-year old acutely developed 2 weeks of fever, emesis and marked malaise while taking tolmetin and methotrexate. She was pale and ill appearing. Her prothrombin time was prolonged. EBV antibody profile was consistent with acute infection, including a positive heterophile antibody; lymphocytosis (22%) evolved. Pulse IV corticosteroids, then IVIG, led to resolution of her illness. Case 2. A 6-year old acutely developed 1 week of fever, abdominal pain, hematemesis, melena and epistaxis while taking ibuprofen. She was irritable and pale, with hepatosplenomegaly and a tender abdomen. Endoscopy revealed a prepyloric ulcer. Serum PCR was positive for EBV. Biopsies revealed EBV hepatitis of liver and rare hemophagocytosis of bone marrow. She received pulse IV corticosteroids, then oral Prednisolone (1mg/kg/day). Due to the risk of viral activation with higher corticosteroid doses, etanercept was added (0.4mg/kg SQ twice per week). Her persistent thrombocytopenia and consumptive coagulopathy resolved, with marked improvement after each etanercept dose; her hepatitis resolved later. This novel description of EBV-associated MAS in oligoarthritis reveals that even the most common category of juvenile idiopathic arthritis is not spared this potentially fatal syndrome.