Familial HLA-B*52 vasculitis: Maternal, atypical cogan’s syndrome with takayasu arteritis-mimicking aortitis and filial Takayasu arteritis

Abstract

Cogan’s syndrome (CS), a rare vasculitis characterized by non-syphilitic, interstitial keratitis and Ménièrelike attacks, is classified into “typical” and “atypical” forms, while Takayasu arteritis (TAK) is a rare large-vessel vasculitis associated with human leukocyte antigen (HLA)-B*52. Very few cases meet both the CS and TAK classification criteria. We herein report a 53-year-old woman diagnosed with atypical CS and aortitis similar to TAK. Her 25-year-old daughter manifested TAK without symptoms of CS, and both are HLA-B*52 positive. Our case highlights the difficulties of distinguishing aortitis with atypical CS from aortitis with TAK.