What is aortitis syndrome?
Aortitis is a form of vasculitis—a family of rare disorders characterized by inflammation of the blood vessels, which can restrict blood flow and damage vital organs and tissues. Aortitis refers to inflammation of the aorta, which carries oxygenated blood from the heart to the rest of the body.
Is Cogan’s syndrome rare?
Cogan syndrome is a rare autoimmune disease that affects the eyes and inner ears. Symptoms of the syndrome include irritation and pain in the eyes, decreased vision, hearing loss , and vertigo.
Is Cogan’s syndrome an autoimmune disease?
Cogan’s syndrome is defined as a chronic inflammatory disease of unknown origin, an autoimmune disease, characterized by bilateral sensorineural hearing loss, vestibular symptoms, inflammatory ocular manifestations with variable risk of developing into a systemic disease.
How do you detect aortitis?
When the condition is caused by an underlying rheumatic condition, patients may experience the following symptoms:
- Headaches.
- Pain in the jaw or tongue muscles when eating or talking.
- Tenderness of the scalp over the temples.
- Loss of vision.
- Double vision.
- Fever.
- Unexplained weight loss.
- Dizziness.
How long can you live with aortitis?
Patients with no complications or with mild to moderately severe complications have a 10-year survival rate of 100% and a 15-year survival rate of 93%-96%. Complications or progression reduce the 15-year survival rate to 66%-68%.
What causes a swollen aorta?
Aneurysms can develop anywhere in the aorta. A thoracic aortic aneurysm is a weakened area in the major blood vessel that feeds blood to the body (aorta). When the aorta is weak, blood pushing against the vessel wall can cause it to bulge like a balloon (aneurysm).
Is Cogan syndrome fatal?
Prognosis. Many people with Cogan’s syndrome have permanent visual or hearing damage. The condition is fatal in less than 10% of patients. However, most patients are able to manage their symptoms and limit complications of the disease with treatment.
How many people have Cogan’s?
Cogan syndrome (CS) prevalence is unknown. To date, approximately 300 cases have been reported. The disease is primarily described in causasians patients with no gender predilection.
Is Cogan syndrome genetic?
The exact cause of Cogan syndrome is unknown, but it’s likely an autoimmune condition. It’s also closely related to vasculitis, which refers to inflammation of your blood vessels. However, researchers aren’t clear on the relationship between the two conditions. There’s no evidence that Cogan syndrome is hereditary.
What is the exact cause of Cogan syndrome?
[1] The exact cause of Cogan syndrome is not well-understood. It is thought that the syndrome is caused by an autoimmune response that causes the immune system to attack the tissues of the eyes and ears. Cogan syndrome is not known to run in families. [2]
What happens if Cogan syndrome is left untreated?
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.
How old do you have to be to have Cogan syndrome?
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.
What are the treatment options for Cogan syndrome?
Cogan syndrome is not known to run in families. Diagnosis of Cogan syndrome is based on observing symptoms associated with the syndrome and ruling out other possible causes of the symptoms. Treatment options generally include corticosteroids and immunosuppressive agents.