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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 initial history and physical exam except for sarcoidosis and syphilis.

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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