Macrophage activation syndrome in children with systemic lupus erythematosus and children with juvenile idiopathic arthritis

Arthritis Rheum. 2012 Dec;64(12):4135-42. doi: 10.1002/art.34661.


Objective: To describe patient demographics, interventions, and outcomes in hospitalized children with macrophage activation syndrome (MAS) complicating systemic lupus erythematosus (SLE) or juvenile idiopathic arthritis (JIA).

Methods: We performed a retrospective cohort study using data recorded in the Pediatric Health Information System (PHIS) database from October 1, 2006 to September 30, 2010. Participants had International Classification of Diseases, Ninth Revision, Clinical Modification diagnosis codes for MAS and either SLE or JIA. The primary outcome was hospital mortality (for the index admission). Secondary outcomes included intensive care unit (ICU) admission, critical care interventions, and medication use.

Results: A total of 121 children at 28 children's hospitals met the inclusion criteria, including 19 children with SLE and 102 children with JIA. The index admission mortality rate was 7% (8 of 121 patients). ICU admission (33%), mechanical ventilation (26%), and inotrope/vasopressor therapy (26%) were common. Compared to children with JIA, those with SLE had a similar mortality rate (6% versus 11%, respectively; exact P = 0.6). More patients with SLE than those with JIA received ICU care (63% versus 27%; P = 0.002), received mechanical ventilation (53% versus 21%; P = 0.003), and had cardiovascular dysfunction (47% versus 23% received inotrope/vasopressor therapy; P = 0.02). Children with SLE and those with 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.

Conclusion: Organ system dysfunction is common in children with rheumatic diseases complicated by MAS, and more organ system support is required in children with underlying SLE than in children with JIA. Current treatment of pediatric MAS varies based on the underlying rheumatic disease.

Publication types

  • Comparative Study
  • Multicenter Study
  • Research Support, N.I.H., Extramural

MeSH terms

  • Adolescent
  • Arthritis, Juvenile / complications*
  • Child
  • Child, Preschool
  • Cohort Studies
  • Cyclophosphamide / therapeutic use
  • Cyclosporine / therapeutic use
  • Female
  • Hospital Mortality
  • Humans
  • Infant
  • Infant, Newborn
  • Inpatients*
  • Intensive Care Units, Pediatric
  • Interleukin-1 / antagonists & inhibitors
  • Lupus Erythematosus, Systemic / complications*
  • Macrophage Activation Syndrome / drug therapy*
  • Macrophage Activation Syndrome / etiology*
  • Macrophage Activation Syndrome / mortality
  • Male
  • Mycophenolic Acid / analogs & derivatives
  • Mycophenolic Acid / therapeutic use
  • Retrospective Studies
  • Treatment Outcome


  • Interleukin-1
  • Cyclosporine
  • Cyclophosphamide
  • Mycophenolic Acid