Macrophage activation syndrome in children with systemic lupus erythematosus and juvenile idiopathic arthritis
Arthritis & Rheumatism, 08/09/2012
Bennett TD et al. – Organ system dysfunction is common in children with rheumatic diseases complicated by macrophage activation syndrome (MAS), and children with underlying systemic lupus erythematosus require more organ system support than children with juvenile idiopathic arthritis. Current treatment of pediatric MAS varies based on the underlying rheumatic disease.Methods
- Retrospective cohort study of the Pediatric Health Information System (PHIS) database, Oct 1, 2006 to September 30, 2010.
- Participants had ICD-9-CM diagnosis codes for MAS and either SLE or JIA.
- The primary outcome was hospital mortality.
- Secondary outcomes included intensive care unit (ICU) admission, critical care interventions, and medication use.
- 121 children at 28 children's hospitals met inclusion criteria, including 19 with SLE and 102 with JIA.
- Index admission mortality was 7% (8/121).
- ICU admission (33%), mechanical ventilation (26%), and inotrope/vasopressor therapy (26%) were common.
- Compared to children with JIA, those with SLE had similar mortality (6% versus 11%, exact p = 0.6), more ICU care (63% versus 27%, p = 0.002), more mechanical ventilation (53% versus 21%, p = 0.003), and more cardiovascular dysfunction (inotrope/vasopressor 47% versus 23%, p = 0.02).
- Children with SLE and JIA received cyclosporine at similar rates, but more children with SLE received cyclophosphamide and mycophenolate mofetil and more children with JIA received interleukin-1 antagonists.