Macrophage activation syndrome in neonates born to mothers with adult-onset Still's disease: Perinatal effect of maternal IL-18

Clin Immunol. 2019 Oct:207:36-39. doi: 10.1016/j.clim.2019.07.005. Epub 2019 Jul 9.

Abstract

Overproduction of interleukin (IL)-18 is closely related to the pathogenesis of adult-onset Still's disease (AOSD) and the development of macrophage activation syndrome (MAS), a life-threating complication of AOSD. We reported three cases of MAS occurring in infants born to mothers with AOSD. The infants developed MAS at age 13 and 8 days and at birth. Serum IL-18 levels were extremely elevated in all infants (147,000 pg/mL; 378,000 pg/mL; 95,000 pg/mL) as well as in their mothers (58,500 pg/mL; 367,000 pg/mL; 84,000 pg/mL). Physicians should be aware that infants born to mothers with AOSD are at a risk of developing MAS. Serum IL-18 levels in mothers with AOSD and their infants should be monitored.

Keywords: Adult-onset Still's disease; Interkeukin-18; Macrophage activation syndrome.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Blood Transfusion
  • Dexamethasone / therapeutic use
  • Female
  • Ferritins / blood
  • Glucocorticoids / therapeutic use
  • Humans
  • Infant, Newborn
  • Interleukin-18 / blood
  • Macrophage Activation Syndrome / etiology*
  • Male
  • Prednisolone / therapeutic use
  • Still's Disease, Adult-Onset / complications*

Substances

  • Glucocorticoids
  • Interleukin-18
  • Dexamethasone
  • Ferritins
  • Prednisolone