Cogans Syndrome - A rare autoimmune-mediated rheumatic disorder of interstitial keratitis
(inflammation of the cornea). Cogan's syndrome develops in children and young adults
shortly after their recovery from an otherwise unremarkable respiratory infection.
The cause is not known. However, one theory is that it is an autoimmune disorder
in which the body's immune system mistakenly attacks tissue in the eye and eye.
The condition may also be associated with blood-vessel inflammation (called vasculitis)
in other areas of the body that can cause major organ damage or, in a small number
of cases, even death.
It most commonly occurs in a person's 20’s or 30’s.
Diagnosis:
A health professional diagnoses Cogan's syndrome after finding the typical combination
of problems associated with this disease in the eye and inner ear. Until both the
eye and the inner ear are affected, the diagnosis may be uncertain. Either area may
be affected first, or the eye and ear problems may develop at the same time.
A health professional will want to rule out an infection (especially syphilis, tuberculosis,
viral infection and chlamydia) or another rheumatic disease (including rheumatoid
arthritis, sarcoidosis and wegener's granulomatosis) as the cause of your symptoms.
A health professional will refer the patient to an ophthalmologist for an eye examination
to look for eye problems, including a condition called interstitial keratitis, which
is inflammation of the cornea, the transparent covering of the eye.
A health professional also will want to evaluate the nervous system (for example,
reflexes, strength and sensation), and check the ears by testing hearing, balance
and inner ear function.
The most common symptoms include:
Red, painful, light-sensitive eyes or blurred vision
Hearing loss, which may become profound and permanent
Vertigo (a sensation of the room spinning; may be called dizziness)
Poor balance
Nausea, vomiting
Fever, fatigue, weight loss
More rarely - enlarged lymph nodes, rash, chest pain, arm pain and shortness of breath.
Cogan's syndrome is a chronic (long-lasting) disease. Symptoms come and go, and respond
well to treatment, but usually the disease is prolonged or even lifelong. There is
no way to prevent Cogan's syndrome. Many people with Cogan's syndrome have permanent
visual or hearing damage. The condition is fatal in less than 10% of people. However,
most are able to manage their symptoms and limit complications of the disease with
treatment..
Treatment
Mild eye disease may be treated with anti-inflammatory medications that are applied
to the eye. If these medications do not work well enough, oral antibiotics may be
recommended in case an infection is causing the eye inflammation.
For more severe disease, oral corticosteroids may be necessary. When large amounts
of steroids are required or if the disease is severe and is not responding to steroid
therapy, other immunosuppressive medications often are recommended. Occasionally,
if the disease has damaged blood vessels, surgery may need to be done to correct
the problem.
If excess fluid in the inner ear is causing balance problems, the health professional
may prescribe diuretic medications, which increase urination and removal of fluid
from the body. A sense of imbalance may be treated with antihistamines or benzodiazepines,
and bed rest.
When hearing is impaired and does not improve with medical treatment, cochlear implants
may be helpful. Cochlear implants are electronic devices that translate sounds into
electrical signals that are transmitted to the brain, bypassing the malfunctioning
part of the ear. Part of the device is implanted in the ear, and part is worn outside
the ear.
If the front of the eye is damaged severely by inflammation, a corneal transplant
may improve vision. A corneal transplant is surgery that replaces the scarred cornea
with one from an organ donor.
