Wednesday

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Wednesday AM report 09-16-09
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Uveitis and Cogans syndrome
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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.

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Uveitis symptoms
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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.

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Diagnosis of uveitis
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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.
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Diagnostic testing
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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

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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.

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Cogans 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.

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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.

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Ocular and Inner Ear disease

• Although interstitial keratitis is classic any
  inflammation can be
• seen in Cogans 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 Cogans syndrome.

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

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Audiogram
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Thank you