Aging Begins at 30

Painful Hat Disease

Ian Maclean Smith, M.D.
Emeritus Professor
Department of Internal Medicine
University of Iowa Hospitals and Clinics
Creation Date: April 2002
Last Revision Date: April 2002
Peer Review Status: Internally Peer Reviewed

The disease is a rarity under 50. Temporal arteritis is inflammation of medium and large sized arteries especially the carotid in the neck and its branches. The temporal artery is a branch of the same artery that goes to the retina. There is a cartilaginous projection in front of your ear hole, and the temporal artery pulse can be felt in front and above traveling across the temple. The inflamed artery is tender, feels nodular and has a reduced or absent pulsation. The area over the blood vessel can be red, swollen and different from the one on the other side. The first patient described in 1890 said, "I've got red streaks on both temples and its painful to wear my hat." He complained that chewing on a tough piece of beef he had no pain to start but the more he chewed the more he had pain in his jaw or tongue and he had to quit eating. That's called jaw claudication. Severe throbbing headaches can occur in both temples but there is no one "arteritis" type of pain. When the doctor dilates the pupils to look at the retina the "disc" where the nerves enter may be pale or swollen or both. A blind spot, not suspected by the patient, may be found by examining the visual fields.

There is an associated disease called polymyalgia rheumatica, which can occur with or without temporal arteritis. These people, again usually elderly, have morning rheumatism about the shoulder girdle or pelvis that may radiate down the limbs.

Because of the dire consequences to sight of a missed diagnosis, doctors are very anxious to spot temporal arteritis promptly. On the other hand, the side effects of high dose prednisone (the only effective treatment) can be severe that it should not be used unnecessarily. To help, Drs. Smetana and Shimerling of the Harvard Medical School searched 34 years of the literature. They found 114 published studies on temporal arteritis. (See JAMA January 2^nd 2002 page 92). Among these were 41 biopsy-proved temporal arteritis cases (certain diagnosis) and cases that had been suspected but proved negative by biopsy. The referring doctors had not done too badly since 39% of the biopsies were positive showing how important biopsies are.

The story that the patients told described jaw claudication or seeing double 3 to 4 times more often in the positive biopsy cases as in the negative ones. But claudication only occurred in 34% and seeing double in 9%. Nothing else helped consistently in the story. Not finding anything wrong when examining the temporal artery was strongly against the diagnosis. Anemia present in 44%, long thought to be helpful, was not. Having a normal sedimentation blood test was strongly against the diagnosis. In fact it was the best clue. The Erythrocyte Sedimentation Rate is the distance red cells fall in anticoagulated blood in one hour. The positives on biopsy averaged 88mm and the negatives 10 mm. The patients negative for arteritis were often sick but with something else such as joint disease especially polymyalgia rheumatica, lymphoma, arteriosclerosis of the carotid artery, diabetes or another kind of optic nerve inflammation.

Over age 50, 24 per 100,000 women and 8 per 100,00 men get temporal arteritis. It is less common in African Americans than in White Americans.

Treatment is with prednisone by mouth 40 to 60 mgms daily tapering to 8 to 10 mgms daily and continuing for 1 to 2 years. Only 1% develop blindness after treatment and if sight loss has started, only 13% go blind.

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See related Patient Topics Bones, Joints and Muscles, Polymyalgia Rheumatica or Skin, Hair and Nails.

See related Provider Topics Bones, Joints and Muscles, Polymyalgia Rheumatica or Skin, Hair and Nails.

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