Macrophage activation syndrome as initial presentation of systemic lupus erythematosus

Pediatr Neonatol. 2008 Apr;49(2):39-42. doi: 10.1016/S1875-9572(08)60010-8.


Macrophage activation syndrome (MAS) is known to be a severe and potentially life-threatening complication of rheumatic disorder, especially systemic juvenille rheumatoid arthritis. It is very rare for MAS to be an initial presentation of systemic lupus erythematosus (SLE). Here, we report a 14-year-old girl in whom MAS developed as an initial presentation of SLE. With early diagnosis and administration of cyclosporine A, she had a fair outcome. Further testing showed positive anti-dsDNA about 8 months later.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Antibodies, Antinuclear / blood
  • Cyclosporine / therapeutic use
  • Female
  • Humans
  • Immune System Diseases / etiology*
  • Lupus Erythematosus, Systemic / complications*
  • Lupus Erythematosus, Systemic / diagnosis
  • Lymphohistiocytosis, Hemophagocytic / etiology
  • Macrophage Activation*
  • Rheumatic Diseases / complications*
  • Syndrome


  • Antibodies, Antinuclear
  • Cyclosporine