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report 09-16-09

Uveitis and

Cogan’s syndrome

Definition of Uvea/Uveitis

Uvea represents the middle portion of the eye (Latin word for grape).

Anterior uveitis synonymous with iritis. In case of involvement of ciliary body = iridocyclitis.

Terms to describe posterior uveitis to the lens include vitritis, intermediate uveitis, pars planitis, choroiditis, retinitis, chorioretinitis or retinochoroiditis.

Uveitis symptoms

Anterior uveitis causes pain and redness in different variations. The degree of vision loss is also variable.

Uveitis symptoms

Posterior uveitis is more likely painless but may result in visual changes such as floaters or reduced visual acuity.

Anterior uveitis is 4 times more likely than posterior uvetitis.

Diagnosis of uveitis

Etiology of uveitis Infections (CMV, toxoplasmosis, syphilis, tuberculosis, cat scratch disease and West Nile and Herpes infections)

Systemic immune-mediated disease (spondylarthritis = typically unilateral uveitis), but also psoriasis and IBD.

Syndromes confined primarily to the eye

Masquerade syndromes (paraneoplastic)

Rule of thumb (according to UpToDate): In general, in patients with uveitis related to a systemic disorder, the associated diagnosis WILL be apparent at the time of initialhistory and physical exam except for sarcoidosis and syphilis.

Diagnostic testing

Diagnostic testing

Taylor the work-up to the HISTORY!

No further work-up needed if first occurrence of unilateral non-granulomatous uveitis and unremarkable history and physical!

Chest x-ray, CBC, ANA, RPR, PPD, Lyme titer

Treatment of uveitis

If systemic or infectious cause was ruled out: Topical glucocorticoids and dilating drops (scopolamine)

If refractory to topical treatment or in case of posterior involvement: Systemic steroids (prednisone = 1mg/kg) for 6-12 months. 2 week course can be considered in HLA-B27 related disease.

Very rarely immunosuppresive agents like (TNF-antagonists) have to be considered.

Cogan’s syndrome

Chronic inflammatory disorder that most commonly affects young adults.

Hallmark is interstitial keratitis (sometimes uveitis) and vestibuloauditory dysfunction.

Pathologic findings are: - lymphocyte and plasma cell infiltration of the spiral ligament - endolymphatic hydrops - degenerative changes in the organ of corti - extensive new bone formation in the inner ear - demyelination and atrophy of the vestibular and cochlear branches of the eighth cranial nerve.

Pathogenesis

Autoimmune disorder but mechanisms are unknown.

1/4 to 1/3 of patients develop syndrome after viral infection.

Definite diagnosis can only be made by characteristic involvement of the eye and inner ear. Less than 5 percent have systemic manifestation like systemic vasculitis.

Ocular and Inner Ear disease

Although interstitial keratitis is classic any inflammation can be seen in Cogan’s disease.

Inner ear manifestations of CS are Meniere-like attacks like vertigo, ataxia, nausea, vomiting, tinnitus and hearing loss. Hearing loss is typically sudden. Down-fluctuation of hearing loss can occur after viral infection. If eye inflammation is present it is most likely due to Cogan’s syndrome.

Treatment can be started with NSAIDS or steroids. In case of cochlear hydrops HCTZ and furosemide can be considered.

Audiogram

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