Atypical Cogan's Syndrome Mimicking Giant Cell Arteritis Successfully Treated with Early Administration of Tocilizumab

Intern Med. 2022 Apr 15;61(8):1265-1270. doi: 10.2169/internalmedicine.7674-21. Epub 2021 Oct 5.

Abstract

A 49-year-old Japanese man with a 2-month history of a fever, headache, and bilateral conjunctival hyperemia was admitted. His condition fulfilled the giant cell arteritis classification criteria (new headache, temporal artery tenderness, elevated ESR) and atypical Cogan's syndrome (CS) with scleritis and sensorineural hearing loss (SNHL). The interleukin (IL)-6 serum level was extremely high. Two weeks after his insufficient response of SNHL and scleritis to oral prednisolone, we administered tocilizumab (TCZ); rapid improvements in scleritis and SNHL occurred. Early IL-6 target therapy can help prevent irreversible CS-induced sensory organ damage.

Keywords: Cogan's syndrome; giant cell arteritis; interleukin-6; tocilizumab.

Publication types

  • Case Reports

MeSH terms

  • Antibodies, Monoclonal, Humanized
  • Apraxias / congenital
  • Cogan Syndrome* / diagnosis
  • Cogan Syndrome* / drug therapy
  • Giant Cell Arteritis* / diagnosis
  • Giant Cell Arteritis* / drug therapy
  • Headache
  • Hearing Loss, Sensorineural* / diagnosis
  • Hearing Loss, Sensorineural* / drug therapy
  • Hearing Loss, Sensorineural* / etiology
  • Humans
  • Keratitis*
  • Male
  • Middle Aged
  • Scleritis*

Substances

  • Antibodies, Monoclonal, Humanized
  • tocilizumab

Supplementary concepts

  • Apraxia, oculomotor, Cogan type