Cogan's syndrome is a new nosological entity in the current classification of systemic vasculitides: A clinical case and a review of literature

Abstract

The paper describes a clinical case of the typical variant of Cogan's syndrome (CS), a new nosological entity in the current classification of systemic vasculitides (SV), which belongs to a group of variable vasculitides. The literature review highlights in detail the problems of the diagnosis and treatment of this rare disease. CS is characterized by inflammatory eye involvement (interstitial keratitis, uveitis, and episcleritis) and hearing problems (sensorineural hearing loss, vestibular disorders) with the possible development of vasculitis at other sites, aortitis, and aortic or mitral lesions. A systemic lesion involving the ears and eyes necessitates to rule out granulomatosis with polyangiitis (Wegener's) and to make a differential diagnosis with a wide range of diseases. The given data underline the need for the interdisciplinary collaboration of rheumatologists, otorhinolaryngologists, audiologists, oculists, and cardiologists in order to improve the diagnosis and treatment of this form of SV.