Clinicopathologic Features of Adult-onset Still's Disease Complicated by Severe Liver Injury

Intern Med. 2024 Feb 15;63(4):503-511. doi: 10.2169/internalmedicine.2043-23. Epub 2023 Jun 21.

Abstract

Adult-onset Still's disease (AOSD) is a systemic inflammatory disorder. Severe liver injury has rarely been reported, although liver enzyme elevation is a common complication of AOSD. We herein report four cases of relapsed AOSD with severe liver disorder by tapering or terminating corticosteroids. Liver specimens revealed robust infiltration of inflammatory cells throughout the lobule, especially cluster of differentiation (CD) 8-positive cells. Relapsed AOSD was refractory to corticosteroid reintroduction and required immunosuppressants. Severe liver injury with AOSD is pathologically characterized by extensive lobular infiltration of CD8-positive cells, and we should consider additive immunosuppressive agents on corticosteroids for treatment.

Keywords: CD8-positive cells; acute liver injury; adult-onset Still's disease.

MeSH terms

  • Adrenal Cortex Hormones / therapeutic use
  • Adult
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Liver
  • Still's Disease, Adult-Onset* / complications
  • Still's Disease, Adult-Onset* / drug therapy

Substances

  • Immunosuppressive Agents
  • Adrenal Cortex Hormones