Isolated Aortitis

Aortitis

Aortitis is a general term which means inflammation of the aorta, the large blood vessel which transports blood from the heart to the body.  The aortic root is the part of the aorta which is directly connected to the left ventricle (chamber) of the heart.  The blood flows from the aorta through its major branches like the carotid arteries to the head, the subclavian arteries to the arms, and iliac arteries to the legs, as well as other branches to organs like the kidneys, spleen and bowel.

Aortitis can cause narrowing (stenosis), leading to reduced blood flow, as well as aneurysm (widening) of the aorta, causing the aorta to rupture.

What causes Aortitis?

Aortitis has a number of causes.  Some cases are “isolated” aortitis, meaning that the aorta is inflamed without any identifiable associated diseases. Aortitis can occur as isolated idiopathic disease of the aorta. A form of aortitis affecting tissues surrounding the aorta can occur with a disease called retroperitoneal fibrosis, or as a result of aneurysms caused by atherosclerosis. Other causes are rheumatic diseases including various forms of vasculitis, as well as infections.

Noninfectious causes of aortitis are forms of large vessel vasculitis, most commonly giant cell arteritis and Takayasu arteritis. Other autoimmune diseases associated with aortitis include rheumatoid arthritis, spondyloarthritis, systemic lupus erythematosus, sarcoidosis, Behçet disease, relapsing polychondritis, ANCA associated vasculitis and Cogan syndrome.

Infectious causes of aortitis include various bacteria, as well as tuberculosis and syphilis.

How common is it?

Isolated aortitis is very rare. There are no reliable figures for how common it is. Most cases are due to a rheumatic cause like giant cell arteritis and Takayasu arteritis.

Who gets Isolated Aortitis?

Aortitis is most commonly described in patients with other rheumatic diseases and forms of vasculitis. Isolated aortitis has been described in older individuals and can affect both men and women.

What are the symptoms?

There are a number of symptoms of aortitis, but none are specific to aortitis and are more commonly associated with other diseases. Many patients have no symptoms at all in the first stages of aortitis. It is not uncommon that inflammation of the aorta is only discovered at the time of surgery for aneurysm.

Some of these symptoms include back pain and abdominal pain, fever, chest pain and shortness of breath with heart failure. Other symptoms may include fatigue, arm or leg weakness or pain with use, lightheadedness, dizziness, fainting and headaches.  Examination may reveal abnormal heart sounds, abnormalities of blood pressure (both high and low), a difference in blood pressure between both arms, reduced or absent pulse in the wrists and sometimes ankles, and anemia.

How is it diagnosed?

The patient’s history, physical examination, laboratory tests and radiographic studies are most helpful. A physical exam may reveal abnormal heart sounds and sounds over the major arteries. There are no specific blood tests, but tests of inflammation like the sedimentation rate (ESR) and the C-reactive protein (CRP) may be abnormally high. Diagnosis is usually made with echocardiography, as well as other techniques like dye based angiography, computed tomographic angiography (CTA) and magnetic resonance angiography (MRA) which reveal abnormalities of the aorta (widening, or aneurysms, and stenosis, or narrowing).

How is it treated?  

The treatment of aortitis depends on the underlying cause. Infections are managed with antibiotic agents appropriate for the identified cause. In cases of isolated aortitis and aortitis associated with other rheumatic diseases and other forms of vasculitis, it is important to get the inflammation under control. Treatments include glucocorticosteroids (usually, prednisone), and cytotoxic drugs like methotrexate, azathioprine, and cyclophosphamide, among others. In some cases, biologics like anti-tumor necrosis factor drugs such as infliximab or rituximab have been used. Isolated aortitis occurring with retroperitoneal fibrosis has been treated with tamoxifen, an anti-estrogen drug with some success.

Surgery is sometimes needed to repair a weak, widened aorta or to relieve blocked arteries.

Care of aortitis requires periodic surveillance of the aorta and large arteries. Vasculitis experts and expert vascular surgeons are important part of the multi-specialty care team. Frequent visits and follow imaging of the heart and aorta may be needed.

What are the complications?

Aortitis of the aortic root can lead to heart failure. Inflammation of the aorta and its major arteries can lead to pain and weakness of the arms and legs with use, kidney failure, stroke and heart attack.

Treatment related complications include side effects of prednisone, such as weight gain due to increased appetite, decreased immune response making infections more likely, increased blood pressure, slower wound healing, emotional fluctuations and hormonal and menstrual cycle changes. Side effects of chemotherapies can include increased risk of infections, anemia, and cancer.

What’s the prognosis?

The outlook for patients with aortitis depends somewhat on the underlying cause. Infectious aortitis has a particularly high mortality rate. The outlook is much better for patients with aortitis associated with rheumatic diseases, and isolated aortitis, particularly when it is diagnosed early. For example, the overall mortality rate for Takayasu over five years is less than 5%. There are few studies of the long term survival rate for isolated aortitis. One of the largest studies of isolated aortitis affecting the aortic root and thoracic aorta revealed a 15 month survival rate of 90%, so that many patients do very well with treatment, including surgical intervention, and follow up. A positive attitude and informed patient are important to the success of treatment. It’s essential to have a good relationship with your treating physicians.

What’s new in Isolated Aortitis?

Research is continuing to better understand the risk factors, causes and treatment of this rare form of vasculitis. More studies are needed to determine the best strategies for detecting and following patients with aortitis.

Revised: May 2013

The Vasculitis Foundation gratefully acknowledges Ds. Kenneth J. Warrington and Dr. Eric L. Matteson from the Mayo Clinic, Rochester, MN for his expertise and contribution in compiling this information.


Click here to get the Isolated Aortitis Brochure in PDF format

 

About Author: joyce

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VCRC-VF Fellowship Program

The Vasculitis Clinical Research Consortium-Vasculitis Foundation Fellowship is a mentored training program of up to two years for physician-investigators who have a strong interest in vasculitis and wish to pursue a period of specialized training with an emphasis on clinical and/or translational patient-oriented clinical investigation.

The trainee will undertake the Fellowship between 2014-2019 at a VCRC-affiliated site in North America that has an established distinct clinical and research program in vasculitis and availability of senior faculty mentors.

"The Vasculitis Foundation is committed to creating greater awareness and interest in vasculitis," said Joyce Kullman, executive director of the VF. "We hope that the VCRC-VF Fellowship will encourage medical professionals to pursue careers in patient care and research linked to vasculitis."

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Medical Institutions

The Vasculitis Foundation encourages vasculitis patients to seek expert medical care for diagnosis and treatment of the disease. The VF works closely with vasculitis experts and centers around the world to ensure patients have access to the most up-to-date medical care.

The Vasculitis Clinical Research Consortium (VCRC) is an integrated group of academic medical centers, patient support organizations, and clinical research resources dedicated to conducting clinical research in different forms of vasculitis and improving the care of patients with vasculitis.

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