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What is Horton’s disease? Symptoms and Treatment

The GCA (sometimes called temporal arteritis or cranial arteritis) denotes an inflammatory disease characterized by inflammation of blood vessels , in particular arteries located at the temples ( temporal arteries ). This disease affects more women over 50 and its origin is poorly known. Drug treatment should be given as soon as the first symptoms appear to avoid complications (aneurysm, blindness, and stroke).

Description

The arteries are responsible for transporting oxygen-rich blood to the various organs. Horton’s disease is defined by inflammation of the arteries, usually the temporal artery located at the temple, which is why we speak of temporal arteritis.

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Populations at risk

Horton’s disease occurs more often in women over the age of 50 (2 to 3 women with men).

What are the risk factors for Horton’s disease?

There are different risk factors:

  • Age (after 50 years);
  • Sex (women);
  • People from Northern Europe and Scandinavia ;
  • People with Forestier-Certonciny syndrome (inflammatory disease of unknown origin also called rhizomelic pseudopolyarthritis);
  • Living in the North: Horton’s disease is more common in Northern Europe.
  • Asian or black people are much less affected by Horton’s disease.

What are the symptoms of Horton’s disease?

Horton’s disease is manifested by:

  • Headaches ( headaches ). These headaches are unusual, sit on the side of the head, at the temples and only one side most often. They are nocturnal and morning. These headaches can also sometimes be at the forehead or be more diffuse.
  • Palpation pain on the path of the arteries located at the temples. These arteries are harder than normal, salient and inflammatory in appearance and the perception of pulsations is diminished.
  • Pain in the jaws or tongue, especially during chewing.
  • Pain in the scalp felt for example when the subject combs or scratches his head.
  • Of vision disorders, as a sudden decrease in visual acuity usually painless, and sometimes transient. There may also be blurred vision, double vision, or other visual disturbances. In case of Horton’s disease, a visual disorder must be treated urgently. Contact a doctor promptly if this happens.
  • An alteration of the general condition, with fatigue, fever, weight loss and lack of appetite.
  • In 40 to 50% of people suffering from Horton’s disease, there is also another disease, rhizomelic pseudoarthritis.
  • At the blood test, numerous abnormalities, in particular an inflammatory syndrome and a high VS (sedimentation rate).
  • Of muscle pain (myalgia).
  • Rigid muscles.

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What are the possible complications of Horton’s disease?

The complications of Horton’s disease are related to a stop of the circulation in the inflammatory arteries. These complications can make the disease serious.
– Ocular complications occur when an artery irrigating the eye is blocked, or when an artery irrigating a nerve of the eye is clogged. Thus 1 to 2% of people with Horton’s disease have a risk of total loss of sight. This is why at the slightest sign of a decline in vision, you must consult urgently.
– The cerebrovascular accident  : it can be transitory (the artery gets clogged then gets out) or not. 5% of people with Horton’s disease have this complication. In case of signs, it is also necessary to consult very quickly.
– psychiatric complications at 3% of patients. There is temporo-spatial disorientation (“I do not know where I am or when”) or mood disorders. This is treated by corticoid treatment.
– Other arterial diseases of the heart, lungs, aorta, upper or lower limbs, kidneys …

How is Horton’s disease diagnosed?

It is not easy to diagnose Horton’s disease at an early stage because the symptoms can direct the doctor to different pathologies. For this reason, the doctor asks the patient several questions in order to rule out certain hypotheses. He asks about the symptoms presented and his background . Then he examines the person who consults with attention, especially his temples.

Blood tests are performed to measure the importance of inflammation ( sedimentation rate ). In addition, the presence of C-reactive protein (PCR) will confirm the existence of inflammation.

The diagnosis can be confirmed by a biopsy  : under local anesthesia, the doctor takes a small fragment of the temporal artery to better analyze it. However, in case of Horton’s disease, the biopsy may be negative because the lesions only exist in places.

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What are the possible treatments for Horton’s disease?

The basic treatment is medicated and consists of a corticosteroid treatment, a cortisone-based treatment. This treatment is very effective, significantly reducing the risk of vascular complications that make the severity of the disease. This treatment works because cortisone is the most powerful anti-inflammatory known, and Horton’s disease is an inflammatory disease. In a week, the improvement is already considerable and in one month of treatment inflammation is normally controlled.
Antiplatelet treatment is added. This is to prevent the platelets in the blood from aggregating and cause a plug blocking the circulation in an artery.
The treatment with cortisone is initially at a dose of attack, then, when the inflammation is mastered (rate of sedimentation or VS returned to normal), the doctor decreases the dose of steroids in steps. It seeks to find the minimum effective dose to limit the adverse effects of treatment. On average, the treatment lasts 2 to 3 years, but it is sometimes possible to stop the cortisone earlier.

Due to the adverse effects that these treatments may cause, people on treatment should be closely monitored during treatment. Special attention should be given to the elderly to prevent an increase in blood pressure ( hypertension ), osteoporosis (bone disease) or eye disease ( glaucoma , cataracts ).
Because of complications related to corticosteroid therapy, alternatives such as methotrexate, azathioprine, synthetic antimalarials, ciclosporin, and anti-TNFα drugs have been studied, but have not been shown to be superior.

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