Publication: Macrophage activation syndrome in an inadequately treated patient with systemic onset juvenile idiopathic arthritis
Date
2009
Journal Title
Journal ISSN
Volume Title
Publisher
Kathmandu University
Abstract
Abstract
Macrophage activation syndrome is a rare and potentially life threatening complication of childhood rheumatic
disorders. It is described most commonly with systemic onset juvenile idiopathic arthritis (soJIA). The major clinical
manifestations are non-remitting fever, hepatosplenomegaly, lymphadenopathy, bleeding diathesis, altered mental status
and rash and may mimic a flare of soJIA. The characteristic laboratory findings are leucopenia, thrombocytopenia and
dramatic elevation of urinary β2 microglobulin. Corticosteroids and cyclosporine are the drugs commonly used in its
management. Early diagnosis and prompt treatment can be life saving. We report a case of 12 year old female child with
inadequately treated systemic onset juvenile idiopathic arthritis who developed fatal macrophage activation syndrome.
The diagnosis and management of macrophage activation syndrome are discussed.
Key words: Macrophage activation syndrome, systemic onset juvenile idiopathic arthritis, leucopenia, children.
Description
Juneja M 1, Jain R 2, Mishra D 3
1Professor, 2Senior Resident, 3Assistant Professor, Department of Pediatrics, Maulana Azad Medical College (University of Delhi), New Delhi, India