Adult-onset Still's disease with macrophage activation syndrome diagnosed and treated based on cytokine profiling: a case-based review

Rheumatol Int. 2020 Jan;40(1):145-152. doi: 10.1007/s00296-019-04446-x. Epub 2019 Sep 20.

Abstract

Adult-onset Still's disease (AOSD) is a relatively rare systemic inflammatory disorder and is diagnosed using various sets of classification criteria, with the Yamaguchi criteria as the most widely used criteria. Herein, we present the case of a 21-year-old woman admitted with a high fever, lasting for over 1 month, who did not fulfill the Yamaguchi criteria. However, by analyzing the inflammatory cytokine profile, we defined this case as AOSD based on a greatly elevated serum interleukin-18 level. In addition, we predicted the occurrence of macrophage activation syndrome by a characteristic increase in the soluble tumor necrosis factor receptor II level, which allowed a timely intervention for this malicious complication. Therefore, we suggest that cytokine profiling will be useful for the diagnosis and management of AOSD.

Keywords: Adult-onset still’s disease; Cytokines; Diagnosis; Macrophage activation syndrome.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Cytokines / immunology
  • Female
  • Glucocorticoids / therapeutic use
  • Humans
  • Interleukin-18 / immunology*
  • Interleukin-6 / immunology
  • Macrophage Activation Syndrome / diagnosis*
  • Macrophage Activation Syndrome / drug therapy
  • Macrophage Activation Syndrome / immunology
  • Methylprednisolone / therapeutic use
  • Neopterin / immunology
  • Prednisolone / therapeutic use
  • Pulse Therapy, Drug
  • Receptors, Tumor Necrosis Factor, Type I / immunology
  • Receptors, Tumor Necrosis Factor, Type II / immunology*
  • Still's Disease, Adult-Onset / diagnosis*
  • Still's Disease, Adult-Onset / drug therapy
  • Still's Disease, Adult-Onset / immunology
  • Young Adult

Substances

  • Cytokines
  • Glucocorticoids
  • Interleukin-18
  • Interleukin-6
  • Receptors, Tumor Necrosis Factor, Type I
  • Receptors, Tumor Necrosis Factor, Type II
  • Neopterin
  • Prednisolone
  • Methylprednisolone