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

Abstract

A 61-year-old man presented with weight loss, bilateral ocular redness, blurred vision, and sensorineural hearing loss. Fluorodeoxyglucose-position emission tomography/computed tomography demonstrated an uptake in the ascending and descending aorta, abdominal aorta and femoral arteries. Atypical Cogan's syndrome complicated with large-vessel vasculitis (LVV) was diagnosed. He was treated with high-dose prednisolone and subcutaneous tocilizumab (162 mg/week), resulting in successful improvements in his ocular and vascular involvements. Although there is currently no established treatment strategy for LVV associated with Cogan's syndrome, our case and literature review suggest that tocilizumab is a viable treatment option for this rare but life-threatening complication.

Cogan’s Syndrome Combined with Hypertrophic Pachymeningitis: A Case Report

“…Studies have signified that TNF antagonists, IL-6 antagonists and Janus kinase inhibitor are effective for some patients. 9 , 13 , 14 …”

“…Studies have signified that TNF antagonists, IL-6 antagonists and Janus kinase inhibitor are effective for some patients. 9,13,14 HP associated with autoimmune diseases can be treated with glucocorticoids and immunosuppressive agents, often requiring glucocorticoid pulse therapy. 15 The doses and duration of glucocorticoid therapy are highly empirical.…”