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 largevessel vasculitis associated with 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-yearold 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.

Ocular Manifestations in Patients with Sensorineural Hearing Loss

“…Systemic features of CS can involve other rheumatologic manifestations, such as vasculitis and aortitis. [146,152] Cases have been reported of patients with CS having aortitis, aortic valve regurgitation, and right coronary artery stenosis. [5,153] Diagnosis of CS is based upon clinical presentation of ocular symptoms and vestibuloauditory dysfunction.…”

“…No preference • Vasculitis [146,152] • Aortitis [5,146,152,153] • Aortic Valve Regurgitation [5,153] • Coronary Artery Stenosis [5,153] • Interstitial keratitis [147] • Iridocyclitis [5,150] • Scleritis [5,150] • Episcleritis [5,150] • Conjunctivitis [5,150] • Retinal artery occlusion [199] • Choroiditis [199] • Retinal hemorrhages [199] • Papilledema [199] • Exophthalmos [199] • SNHL [5,6,11] Table 1. Summary of syndromes that include both ocular and sensorineural hearing loss manifestations.…”

Cogan Syndrome with Aortic Regurgitation and Multiple Vasculopathy

“…The histopathologic microphotograph derived from the resected aortic valves exhibited prominent myxomatous degeneration ( Picture 4 ), although this finding was not specific to CS. CS is a rare and chronic vasculitis, characterized by keratitis, scleritis, arteritis, and valvulitis ( 1 ). Our observations suggest that cardiovascular diseases should be meticulously evaluated as possible comorbidities of CS, as a delayed diagnosis can be fatal.…”