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Temporal arteritis is a poorly understood but very treatable disease. In this condition, inflammation causes medium-sized arteries to become narrow, restricting blood flow to some parts of the body. The arteries most commonly affected are inside the forehead (though not in the brain). Because symptoms are felt in the temples, it's called temporal arteritis, or sometimes cranial arteritis.
However, the same syndrome can occur in other arteries elsewhere in the body, so it is also known as giant cell arteritis. Giant cells are immune cells found in high concentrations inside affected arteries.
Temporal arteritis is almost unheard of in people under 50 years of age. Among people over 50, it strikes as many as 1 in 1,000 people in Canada. Temporal arteritis is more common in women than men. People of African descent are rarely affected.
Temporal arteritis is closely associated with a disease called polymyalgia rheumatica, which many experts believe is a different manifestation of the same underlying disorder. More than 40% of people with temporal arteritis also have the symptoms of polymyalgia rheumatica. Fortunately, the same treatment is effective for both conditions.
Temporal arteritis is an autoimmune disease in which the body's immune system mistakenly attacks human tissue. Temporal arteritis is more common in women than in men.
Autoimmune diseases in general are poorly understood. They tend to run in families, but not so much that we can say they're inherited or genetic diseases. Many doctors believe it takes a combination of predisposing genes and environmental factors to trigger the condition.
The early symptoms of temporal arteritis may be similar to a case of influenza (the flu), with fever, weakness, and possibly weight loss. These symptoms may go on to the classic symptoms of temporal arteritis:
- double vision or blurred vision due to narrowing of the main artery to the eye
- headache in the temple or back of the head
- sensitive, tender scalp
- swollen bumpy arteries in the temple, often lacking a noticeable pulse
- weak or painful jaw muscles - lack of oxygen to the muscles of the jaw makes chewing, talking, and swallowing very painful
- weight loss
If arteritis is left untreated, it can progress to the following symptoms:
- blind spots
- sudden blindness in one eye
Blindness is the most serious threat in arteritis. Other complications such as stroke occur very rarely. In a few cases, vision loss is the first symptom of temporal arteritis. Unfortunately, any damage already done is usually irreversible. Most people get warning symptoms (called prodromes) that give them time to take action. Apart from the flu-like prodrome and the headaches, one of the most common warning signs is polymyalgia rheumatica.
About 15% of people with polymyalgia rheumatica go on to develop temporal arteritis. Arteritis may appear before, during, or after the onset of polymyalgia rheumatica. The primary symptoms of polymyalgia rheumatica are stiffness and pain in the neck, shoulder, and hip muscles, which is often at its worst in the morning. There may also be flu-like symptoms.
Temporal arteritis is diagnosed by a thorough physical examination, blood tests, and sometimes by taking a biopsy (tissue sample) of the affected arteries. Blood tests usually reveal the presence of inflammation in the body.
Two tests, the erythrocyte sedimentation rate (ESR) and C-reactive protein, are usually elevated in temporal arteritis. The ESR measures how fast erythrocytes (red blood cells) settle to the bottom of a thin test tube.
If a biopsy is needed, usually a couple of centimetres are removed from the temporal artery, sometimes from both sides of the forehead. Taking two samples can give a more reliable diagnosis. It's important to distinguish arteritis from the heart diseases that are more usually responsible for narrowing of the arteries, because the treatments are quite different.
The good news about temporal arteritis is that it's highly treatable, and blindness can be avoided. Current treatment will stop eye damage, and eventually will drive away the other symptoms, too. The medication of usually used is prednisone*, a potent corticosteroid that depresses immune system activity. This medication helps to resolve headache, eye, and heart problems associated with temporal arteritis.
Usually, a high dose is given for 2 or 3 weeks to bring the inflammation in the arteries under control. This is then tapered over a month or two. Long-term use of corticosteroids increases the risk of infections and other side effects, so usually the recommended duration of corticosteroid treatment is the shortest one possible that will produce the best results.
Some people need to keep taking low doses for a year or two before their doctor can be sure that symptoms won't return. Others may need to take prednisone for 5 to 10 years or indefinitely. Sometimes, symptoms recur a few times early in treatment and the dosage has to be temporarily raised. Temporal arteritis hardly ever recurs after treatment is finally stopped.
The following side effects are associated with corticosteroid use, but they are much less likely with the lower doses given for temporal arteritis after the first few weeks:
- diabetes
- easy bruising
- fluid retention and weight gain
- glaucoma
- higher blood pressure
- osteoporosis
- rounding of the facial features
- slower healing of wounds
- stomach irritation
Prednisone is also used to treat polymyalgia rheumatica, eliminating symptoms within a day or two of the first dose.
An immunosuppressant medication such as azathioprine or methotrexate may be used as an alternative if prednisone is not tolerated.
Low-dose (81 mg daily) acetylsalicylic acid (ASA) may also be prescribed to help lower the risk of blindness or stroke. These may result if the blood flow carrying oxygen to the optic nerve or to the brain is cut off due to the arteritis.
There's no easy way to prevent arteritis, but several studies have shown a link between vascular (blood vessel) diseases of the eye and diets high in animal fats, salt, and sugar. Smoking almost certainly doesn't help either.
Above all, see a doctor if you have the symptoms listed in the "Symptoms and Complications" section. As many as 1 in 5 people with this disease will go blind without treatment, yet a simple visit to the doctor can prevent any eye damage. You should also get your eyesight checked every 2 years if you're over 50 years of age, and every year if you have diabetes.
*All medications have both common (generic) and brand names. The brand name is what a specific manufacturer calls the product (e.g., Tylenol®). The common name is the medical name for the medication (e.g., acetaminophen). A medication may have many brand names, but only one common name. This article lists medications by their common names. For more information on brand names, speak with your doctor or pharmacist.
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