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

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.

Atypical Cogan's Syndrome with Large-vessel Vasculitis Successfully Treated with Tocilizumab

“…Our case is the fourth case of Cogan's syndrome to be treated with tocilizumab. As shown in Table, we reviewed our case and three reported cases of LVV in Cogan's syndrome that were treated by tocilizumab (12)(13)(14). One case (13) was excluded due to a lack of details concerning the clinical information.…”