TAFRO Syndrome That Responded to Prednisolone-only Treatment: Evaluating Changes in IL-6

Intern Med. 2022 Oct 1;61(19):2967-2972. doi: 10.2169/internalmedicine.9160-21. Epub 2022 Feb 26.

Abstract

Thrombocytopenia, anasarca, fever, reticulin fibrosis, and organomegaly (TAFRO) syndrome is a systemic inflammatory disorder characterized by the above-mentioned symptoms. Because of the similarity in phenotypes between TAFRO syndrome and decompensated liver cirrhosis, an accurate diagnosis is often difficult. We herein report a 62-year-old Japanese patient with TAFRO syndrome who was misdiagnosed with intractable ascites associated with liver cirrhosis. Improvement of symptoms after treatment with prednisolone was associated with interleukin-6 rather than C-reactive protein. The pathogenesis of TAFRO syndrome, which has similar clinical manifestations to liver cirrhosis, remains unclear, and our findings may help elucidate the concept of this condition.

Keywords: Castleman's disease; IL-6; TAFRO syndrome; glomeruloid hemangioma; intractable ascites.

MeSH terms

  • C-Reactive Protein
  • Castleman Disease* / complications
  • Castleman Disease* / diagnosis
  • Castleman Disease* / drug therapy
  • Edema / diagnosis
  • Humans
  • Interleukin-6*
  • Liver Cirrhosis / complications
  • Liver Cirrhosis / drug therapy
  • Prednisolone / therapeutic use
  • Reticulin

Substances

  • Interleukin-6
  • Reticulin
  • C-Reactive Protein
  • Prednisolone

Supplementary concepts

  • Multi-centric Castleman's Disease