PT  - JOURNAL ARTICLE
AU  - Ricardo A G Russo
AU  - Sergio D Rosenzweig
AU  - María M Katsicas
TI  - Hepatitis A-associated macrophage activation syndrome in children with systemic juvenile idiopathic arthritis: report of 2 cases.
DP  - 2008 Jan 01
TA  - The Journal of Rheumatology
PG  - 166--168
VI  - 35
IP  - 1
4099  - http://www.jrheum.org/content/35/1/166.short
4100  - http://www.jrheum.org/content/35/1/166.full
SO  - J Rheumatol2008 Jan 01; 35
AB  - OBJECTIVE: 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.