Severe and life-threatening onset of systemic lupus erythematosus

Reumatol Clin (Engl Ed). 2023 Aug-Sep;19(7):402-403. doi: 10.1016/j.reumae.2022.09.003.

Abstract

Macrophage activation syndrome (MAS) is a potentially life-threatening complication of rheumatic diseases. We report a unique case of a previously healthy 20-year-old female presenting with MAS as first presentation of systemic lupus erythematosus. Remission was achieved with hydroxychloroquine, intravenous methylprednisolone pulse followed by oral prednisolone and cyclosporine. However, the management of MAS is still challenging, and the mortality rate remains high.

Keywords: Ciclosporina; Cyclosporin; Lupus eritematoso sistémico; Macrophagic activation syndrome; Systemic lupus erythematosus; Síndrome de activación macrofágica.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Cyclosporine / therapeutic use
  • Female
  • Humans
  • Hydroxychloroquine / therapeutic use
  • Lupus Erythematosus, Systemic* / complications
  • Lupus Erythematosus, Systemic* / diagnosis
  • Lupus Erythematosus, Systemic* / drug therapy
  • Macrophage Activation Syndrome* / complications
  • Methylprednisolone / therapeutic use
  • Young Adult

Substances

  • Cyclosporine
  • Methylprednisolone
  • Hydroxychloroquine