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Cogan syndrome

Cogan syndrome is rare autoimmune-mediated rheumatic disorder characterized by recurrent cornea inflammation, fever, weight loss and hearing loss. It can lead the patient to blindness or deafness if not treated properly. In 1945 D.G. Cogan first described this term. People of 20s, 30s and children are mostly affected by Cogan syndrome.

What causes Cogan syndrome?

The exact cause of cogan syndrome is still unknown, but thought that it is an autoimmune disorder. Patients own antibody to inner ear and eye tissue causes the eye and ear inflammation. Infection with the bacteria Chlamydia pneumoniae has been demonstrated in some patients prior to the development of Cogan’s syndrome but not proved at.

 

What are the symptoms of cogan syndrome?

  1. Recurrent eye inflammation, including ketatitis, iritis, scleritis, conjunctivitis.
  2. Vision difficulty
  3. Dizziness ,tinnitus , hearing loss, vertigo, poor balance
  4. Vasculitis
  5. fever
  6. Fatigue, weight loss
  7. Nausea and vomiting
  8. Photo sensitivity, rash
  9. enlarged lymph nodes
  10. Chest pain, arm pain, shortness of breath, night sweat.
  11. aortitis, aortic valve insufficiency, pleuritis, pericardial effusion, coronary arteritis, and possibly myocardial infarction.

 

How is cogan syndrome diagnosed?

Felty’s syndrome is diagnosed by patient’s history, physical examination and lab tests.

Lab tests shows:

  1. Increased CRP, WBC and C – reactive protein.
  2. present of anemia or thrombocytopenia
  3. MRI shows abnormal tissue.
  4. Present of antigen in inner ear.

Cogan syndrome can occur in children, and is particularly difficult to recognize in that situation.

How to managed cogan syndrome ?

Anti-inflammatory eye and ear drops are used is mild cases. For moderate cases antibiotic drops can be used. For more severe disease, oral corticosteroids may be necessary to reduce the inflammatory response. When large amounts of steroids are required or if the disease is severe and is not responding to steroid therapy, other immunosuppressive medications like Methotrexate, cyclophosphamide, cyclosporine, and azathioprine are recommended. In some severe cases surgical repair of eye may be done .

References:

  1. A Clinician’s Pearls & Myths in Rheumatology
  2. Medical physiology, Lippincott Williams & Wilkins 3rd edi.
  3. Harrison’s Principles of Internal Medicine, 17th edition.
  4. Davidson’s Principles and Practice of Medicine, 20th Edition
  5. Harrison’s Rheumatology, Second Edition

 

From: Emedicinezone.com

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