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Cogan Syndrome

(Cogan's Syndrome)

By Melvin I. Roat, MD, FACS, Jefferson Medical College, Thomas Jefferson University

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Cogan syndrome is a rare autoimmune disease involving the eye and the inner ear.

Cogan syndrome affects young adults, with 80% of patients between 14 yr and 47 yr. The disease appears to result from an autoimmune reaction directed against an unknown common autoantigen in the cornea and inner ear. About 10 to 30% of patients also have severe systemic vasculitis, which may include life-threatening aortitis.

Symptoms and Signs

The presenting symptoms involve the ocular system in 38% of patients, the vestibuloauditory system in 46%, and both in 15%. By 5 mo, 75% of patients have both ocular and vestibuloauditory symptoms. Nonspecific systemic complaints include fever, headache, joint pain, and myalgia.

Ocular

Ocular involvement includes any combination of the following:

  • Bilateral interstitial keratitis or other corneal stromal keratitis

  • Episcleritis or scleritis

  • Uveitis

  • Papillitis

  • Other orbital inflammation (eg, vitritis, choroiditis)

Ocular symptoms include irritation, pain, photophobia, and decreased vision. Ocular examination shows a patchy corneal stromal infiltrate typical of interstitial keratitis (see Interstitial Keratitis ), ocular redness, optic nerve edema, proptosis, or a combination of these symptoms.

Vestibuloauditory

Vestibuloauditory symptoms include sensorineural hearing loss, tinnitus, and vertigo.

Vascular

A diastolic heart murmur may be present when aortitis is significant. Claudication may be present if limb vessels are affected.

Diagnosis

Diagnosis is based on clinical findings and exclusion of other causes (eg, syphilis, Lyme disease, Epstein-Barr virus infection) by appropriate serologic tests. Urgent evaluation by an ophthalmologist and otolaryngologist is indicated.

Treatment

  • Initially topical and sometimes systemic corticosteroids for ocular involvement

Untreated disease may lead to corneal scarring and vision loss and, in 60 to 80% of patients, permanent hearing loss. Keratitis, episcleritis, and anterior uveitis can usually be treated with topical prednisolone acetate 1% q 1 h to qid. To treat deeper ocular inflammation and especially to treat vestibuloauditory symptoms before they become permanent, prednisone 1 mg/kg po once/day is begun as soon as possible and continued for 2 to 6 mo. Some clinicians add cyclophosphamide, methotrexate, or cyclosporine for recalcitrant cases.

Drugs Mentioned In This Article

  • Drug Name
    Select Trade
  • NEORAL, SANDIMMUNE
  • ORAPRED, PRELONE
  • RAYOS
  • OTREXUP
  • CYTOXAN (LYOPHILIZED)

* This is the Professional Version. *