Arteritis

Arteritis is an inflammatory disease of the body’s arteries. This condition sometimes develops into thrombosis, or the formation of blood clots, which can be fatal. Arteritis is a subtype of vasculitis, a group of conditions that involve inflammation of the blood vessels, which can destroy the blood vessels through chronic inflammation (by thickening them, making them weak, narrow, scarred, and blocked). Giant cell arteritis is a type of arteritis that affects medium and large blood vessels, especially in the head region; this disease is classified as a large-vessel vasculitis in medical classification systems such as the World Health Organization’s International Classification of Diseases, 11th Revision (ICD-11).

Early History

The most common subset of arteritis, giant cell arteritis, was first studied in the 1890s, with systematic studies that started in 1930 helping establish it as a distinct entity. The subsequent decades brought a greater understanding of the disease. Many of its associated symptoms, presentations, and complications were not accurately recorded until the 1950s and 1960s. The debate about whether arteritis and giant cell arteritis were conditions related to vasculitis raged until the 1970s. Eventually, experts concluded that arteritis and giant cell arteritis were both subtypes of vasculitis.

Giant Cell Arteritis

Giant cell arteritis, also called temporal arteritis, is a condition in which the blood vessels (usually the medium and large arteries) leading to the head and brain become damaged or inflamed, but it can also involve the neck, arms, chest, and aorta. It is most common in people over the age of fifty and is more common in women than men. The disease is extremely rare in individuals younger than 50.

Giant cell arteritis usually develops in the arteries surrounding the temporal region of the head (either side of the forehead). This is why it is sometimes called temporal arteritis. However, the name is slightly misleading because the disease can occur in any major blood vessel leading to the brain, not just the temporal region.

Giant cell arteritis causes inflammation and swelling in the lining of arteries. This causes the artery to constrict, severely restricting the blood that flows through that passage. Because blood carries oxygen and other vital nutrients throughout the body, any cause of restricted blood flow can be very serious.

The exact cause of giant cell arteritis remains unknown, although many experts suspect the disease may be an autoimmune disorder, or a disease that causes the body’s immune system to attack its healthy cells. Certain individuals may also be genetically predisposed to the condition. Because so little is understood about the origins of the condition, there is no known way to prevent it from occurring.

Giant cell arteritis may cause a number of serious complications. These include inflammation of the blood vessels, damage to other blood vessels, and the development of aneurysms, or ballooning and bursting of the artery. Other complications include weak eye muscles, blindness, and stroke. Blindness resulting from arteritis may be permanent, and both stroke and aneurysms can be fatal. Anyone who suspects they may suffer from giant cell arteritis should inform a doctor as soon as possible.

A variety of symptoms occur in giant cell arteritis. Doctors may recognize the disease when the patient reports a consistent, throbbing headache originating in the temples, jaw pain, fever, shoulder and hip pain or stiffness, fatigue, tenderness in the scalp, double vision, or loss of vision in one eye.

Because many of the symptoms are common to a number of disorders, doctors may conduct a variety of tests to confirm the presence of giant cell arteritis. These include key tests such as a C-reactive protein test to check for internal inflammation and an erythrocyte sedimentation rate test to check for internal inflammation, along with other general blood tests such as a hemoglobin test to measure the amount of oxygen-carrying protein in the patient’s blood, a liver function test to check liver performance, and a hematocrit test to check red blood cell count (as percentage of total blood volume). If these tests indicate giant cell arteritis, the doctor may order an ultrasound on the affected areas. Ultrasound allows the doctor to study an image of the inflamed arteries without surgical intervention. Should an ultrasound prove inconclusive, the doctor may order a biopsy of the affected area. A biopsy will provide strong evidence if inflammation of the arteries is responsible for the patient’s symptoms, but a negative result cannot always rule out its minimal presence. Advanced imaging techniques such as magnetic resonance imaging (MRI) and positron emission tomography (PET) scans are also increasingly used to detect inflammation in large blood vessels.

Treatment of Arteritis

There is no cure for arteritis. Treatments are geared toward minimizing tissue damage caused by inadequate blood flow, reducing inflammation, and increasing blood flow.

The most common treatment for arteritis is oral corticosteroids. While effective, these medications may cause osteoporosis, high blood pressure, eye problems, weight gain, skin sensitivity, sleeping difficulties, and a weakened immune system. Other treatments may include an aspirin regimen, bone density screenings, and calcium or vitamin D supplements. Treatment regimens may last as long as two years but may extend longer depending on the response to treatment and symptoms. Biologic medications such as tocilizumab may also be used to reduce inflammation and decrease reliance on long-term corticosteroid therapy.

The earlier arteritis is detected, the more likely it is that a patient can lead a long and normal life. Prolonged arteritis causes permanent damage to blood vessels throughout the body, drastically increasing the risk of complications. However, if treated early, the disease can usually be successfully managed.

Other Forms of Vasculitis

Vasculitis is any disease that involves the inflammation of the body’s blood vessels. Some of the many forms of vasculitis include Behçet’s disease, Buerger’s disease, cryoglobulinemic vasculitis, Takayasu arteritis, Kawasaki disease, and IgA vasculitis (formerly Henoch-Schönlein purpura).

Most forms of vasculitis are rare. Some affect only small areas of the body, while others affect entire organ systems. Additionally, while some types of vasculitis last only a short period of time, others may last for the rest of the individual’s life.

Bibliography

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