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Wednesday
AM
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
Uveitis symptoms
 Anterior uveitis causes pain and redness in different variations.
The degree of vision loss is also variable.
 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
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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