Inflammatory Aortic Disease (Aortitis)
Overview
The aorta is the largest artery in the body. It rises from the heart's left ventricle (the major chamber that pumps blood out of the heart) and is filled with oxygen-rich blood that travels throughout the body. When this artery becomes inflamed, the condition is known as aortitis.
Aortitis can develop from three different circumstances:
- An underlying condition that causes inflammation, such as giant cell arteritis (GCA)
- Infection such as salmonella
- Isolated aortitis, which has no underlying cause of the inflammation.
The most common causes of aortitis are GCA and another inflammatory disease (rheumatologic) known as Takayasu's arteritis.
Symptoms
Symptoms of aortitis can vary depending on the location and cause of the inflammation. However, general symptoms may include:
- Back pain
- Abdominal pain
- The aortic valve not closing properly, allowing a backflow of blood (aortic insufficiency)
- Thoracic aortic aneurysm
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
- Difficulty with coordination
- Difficulty with balance
- Night sweats
- Joint pain
- Fatigue
If the condition is caused by an infection, symptoms can be similar to those above and may also include an elevated white blood cell count (leukocytosis).
Causes and Risk Factors
Because the condition can develop from a variety of causes, it can affect both men and women of any age. Most cases of aortitis are associated with GCA and Takayasu's arteritis. Other conditions associated with aortitis are:
- Systemic lupus erythematosus
- Rheumatoid arthritis
- The HLA-B27–associated spondyloarthropathies
- Antineutrophil cytoplasmic antibody–associated vasculitides
- Behçet's disease
- Cogan syndrome
- Sarcoidosis
Infections associated with the condition include:
- Tuberculosis
- Syphilis
- Salmonella
- Other bacteria
Diagnosis
Diagnosis of aortitis will begin with the physician performing a physical examination and recording the patient's medical history. Based on the physical exam, medical history and findings, the medical team may perform a biopsy of the area if they suspect the patient to have GCA. This is the best way to diagnose GCA. During the procedure, a small amount of the temporal artery will be removed using a needle. More than one sample may be taken because the condition does not occur everywhere within the artery. This sample is then examined under a microscope to see if the cells are abnormally large, indicating inflammation.
To check for Takayasu's arteritis, the physician will check for the blood pressure rate in the involved arteries arising from the aorta. In Takayasu's arteritis, pulses and blood pressure will be low or impossible to find. By contrast, there will be generally brisk pulses in the legs. Bruits may be heard over partially narrowed arteries.
Imaging tests may be done, such as magnetic resonance angiography (MRA), Doppler ultrasound and positron emission tomography (PET) to look at the blood vessels and the blood flow through them. These imaging tests will also highlight any areas of inflammation.
Although the condition is generally diagnosed based on the symptoms the patient is experiencing and the aortic images, blood samples may also be used to look for underlying conditions or infections, organ function and other indicators.
Treatment
Treatment of aortitis will depend on the cause of the condition. For infectious aortitis, swift treatment of the underlying infection is very important. Treatment for this type of aortitis will usually begin with broad-spectrum antibiotics given intravenously. Patients may also need surgical treatment to repair any aneurysms that have developed and to remove any damaged or dead tissue.
When an underlying inflammatory disease such as GCA or Takayasu's arteritis causes the condition, treatment will usually focus on relieving symptoms and preventing tissue damage. A corticosteroid such as prednisone is usually given to reduce inflammation, and treatment can take two years or longer. Surgery may also be needed but is often performed at a later time if the condition causes damage.
The knowledgeable and highly trained staff at the Cedars-Sinai Heart Institute will work with each patient to determine the best treatment option.
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