Cogan’S Syndrome

(Cogan syndrome)

Cogan’s syndrome is a rare autoimmune disease of the eye and the inner ear.

Cogan’s syndrome occurs in young adults and 80% of patients are between 14 and 47 years old. The disease appears to be caused by an autoimmune reaction is directed against an unknown autoantigen, which can be found in the cornea and the inner ear. Approximately 10-30% of patients also have severe systemic vasculitis, which may include a life-threatening Aortitis.

Cogan’s syndrome is a rare autoimmune disease of the eye and the inner ear. Cogan’s syndrome occurs in young adults and 80% of patients are between 14 and 47 years old. The disease appears to be caused by an autoimmune reaction is directed against an unknown autoantigen, which can be found in the cornea and the inner ear. Approximately 10-30% of patients also have severe systemic vasculitis, which may include a life-threatening Aortitis. The main symptoms symptoms and complaints relate to 38% of the patient’s ocular system, in 46%, the equilibrium system, and in 15% both. After 5 months 75% of patients have both ocular and vestibulocochlear symptoms. Nonspecific symptoms include fever, headache, joint pain and myalgia. Ocular symptoms Ocular involvement includes any combination of the following: Bilateral interstitial keratitis or other stromal keratitis episcleritis or scleritis Uveitis Papillitis Other orbital inflammation (. Eg Vitritis, choroiditis) Eye symptoms are irritation, pain, photophobia, and visual loss. The ocular examination shows a blotchy, stromal corneal infiltrate that for interstitial keratitis is typical (interstitial keratitis), eye redness, Optikusödem, proptosis or a combination of these Symptome.Vestibulocochlear vestibulocochlear symptoms include sensorineural hearing loss, tinnitus and Vertigo.Vaskuläre symptoms If the Aortitis is significant , a diastolic murmur may be present. Claudication may be present if extremities vessels are affected. Diagnosis Diagnosis is based on clinical findings and the exclusion of other causes (eg. As syphilis, Lyme disease, Epstein-Barr virus infection) by appropriate serological tests. An urgent ophthalmic and ENT medical examination is indicated. Therapy First topical and sometimes systemic corticosteroids at involvement of the eyes untreated, the disease can lead to corneal scarring and vision loss, and in 60-80% of patients in permanent hearing loss. Keratitis, episcleritis and anterior uveitis can be treated per hour four times a day usually with topical prednisolone acetate 1%. To treat deeper eye infections and, in particular, to treat vestibulocochlear symptoms before they become permanent is with prednisone 1 mg / kg p.o. 1 time / day started as soon as possible and continued for 2-6 months. Some clinicians add in stubborn cases still cyclophosphamide, methotrexate or cyclosporine added.

Health Life Media Team

Leave a Reply