K53
OCULAR MANIFESTATIONS IN COGAN-SYNDROME I
M. Zierhut, T. Schlote, T. Daikeler, N. Stübiger, I. Kötter, D. Bless
Cogan-Syndrome I is an uncommon inflammatory disease of unknown etiology involving the eye and ear. The most prominent ocular sign is interstitial keratitis.
Methods: Retrospectively the charts of ten patients (9 female, 1 male, age ranged from 18 to 78 years, median 32,5 y) with Cogan-Syndrome I, who were treated in the department of internal medicine, ophthalmology and otolaryngology between 1978 and 1996, were investigated.
Results: First ocular manifestations were: episcleritis (2), nodular scleritis (1), necrotising scleritis with keratitis (1), panuveitis (1), anterior uveitis (1), posterior uveitis (1), interstitial keratitis (2) and keratouveitis (1). In 9 patients both eyes were affected. The the course of the disease additional ocular symptoms occured: panuveitis (1), episcleritis (1), keratitis (1) and anterior uveitis (1). All patients had severe audiovestibular dysfunction. Additional systemic findings
were: polyneuropathy (1), pericarditis with arthritis and polyserositis (1), fibromyalgia (1) and suspected cerebral vasculitis (1). Four patients received systemic corticosteriods alone and four patients azathioprine and steroids treating audiovestibular dysfunction. Both patients with scleritis (one associated with panuveitis) showed a severe course with visual impairment despite the use of metotrexate in one and cyclophosphamide in the other case. Visual acuity was unaffected in the remaining 8 patients. Audiovestibular function improved in only three cases.Conclusions: Episcleritis, scleritis and uveitis are common manifestations of Cogan-Syndrome I. Whereas ear manifestations had a bad prognosis, visual prognosis is mostly excellent, except for severe cases of scleritis and panuveitis.
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