Uveitis/Cogan`s Syndrome
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Transcript Uveitis/Cogan`s Syndrome
Wednesday
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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
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
Thank you