Hepatitis A-Associated Macrophage Activation Syndrome in Children with Systemic Juvenile Idiopathic Arthritis: Report of 2 Cases
RICARDO A.G. RUSSO, SERGIO D. ROSENZWEIG, and MARÍA M. KATSICAS
ABSTRACT.
We describe two 3-year-old patients with systemic juvenile idiopathic arthritis (SJIA) who developed hepatitis A-associated macrophage activation syndrome (MAS). One patient showed MAS as the presenting manifestation of SJIA, while MAS complicated SJIA during the second year of the disease course in the other child. Both girls presented with fever, jaundice, hepatosplenomegaly, neurological involvement, mucosal hemorrhage, and purpura. Cytopenias, hypofibrinogenemia, and hemophagocytosis confirmed the diagnosis. After aggressive treatment with high-dose corticosteroids and immunosuppressants one patient entered remission while the other one died. Hepatitis A virus may induce severe MAS in SJIA. (First Release Nov 15 2007; J Rheumatol 2008;35:166-8)
Key Indexing Terms:
MACROPHAGE ACTIVATION SYNDROME
JUVENILE IDIOPATHIC ARTHRITIS
HEPATITIS A
HEMOPHAGOCYTOSIS
From Servicio de Inmunología y Reumatología, Hospital de Pediatría "Prof. Dr. Juan P. Garrahan," Buenos Aires, Argentina.
R.A.G. Russo, MD; S.D. Rosenzweig, MD, PhD; M.M. Katsicas, MD.
Address reprint requests to Dr. R.A.G. Russo, Servicio de Inmunología y Reumatología, Hospital de Pediatría "Prof. Dr. Juan P. Garrahan," Pichincha 1880, 1245 Buenos Aires, Argentina.
Accepted for publication September 20, 2007.
