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Cogan Syndrome

(Cogan's Syndrome)

By

Melvin I. Roat

, MD, FACS, Sidney Kimmel Medical College at Thomas Jefferson University

Last full review/revision May 2020| Content last modified May 2020
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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 and 47 years. 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 months, 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

  • 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, 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

  • Clinical evaluation

Diagnosis is based on clinical findings and exclusion of other causes of stromal keratitis (eg, syphilis, Lyme disease, Epstein-Barr virus infection) by appropriate serologic tests. Antibodies to inner ear antigens, anti-Hsp70 (associated with sensorineural hearing loss), and antineutrophil cytoplasmic antibodies (associated with vasculitis) have been found in patients with Cogan syndrome. Urgent evaluation by an ophthalmologist and otolaryngologist is indicated when Cogan syndrome is suspected.

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 (and symptoms of corneal involvement), episcleritis, and anterior uveitis can usually be treated with topical prednisolone acetate 1% every hour up to 4 times a day for 2 to 3 weeks. To treat deeper ocular inflammation and especially to treat vestibuloauditory symptoms before they become permanent, prednisone 1 mg/kg orally once a day is begun as soon as possible and continued for 2 to 6 months. Some clinicians add cyclophosphamide, methotrexate, cyclosporine, or infliximab for recalcitrant cases.

Key Points

  • Cogan syndrome is a rare autoimmune syndrome affecting mainly the ocular and vestibuloauditory systems.

  • Life-threatening aortitis sometimes occurs.

  • If untreated, permanent vision and hearing loss can result.

  • Urgent evaluation by an ophthalmologist and otolaryngologist is indicated.

  • Treatment is typically with corticosteroids (topical and systemic).

Drugs Mentioned In This Article

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