Giant cell arteritis, also known as Horton's disease or temporal arteritis, is the most common primary vasculitis, affecting 200 people per million per year. The disease affects various vessels, including those that supply the eye, which entails the risk of blindness. What are the causes and symptoms of Horton's disease? What is its treatment.

Giant cell arteritis , otherwiseHorton's diseaseortemporal artery inflammation(Latinarteritis gigantocellularis, arteritis temporalis , giant cell arteritis abbreviated as GCA, temporal arteritis) is a disease whose essence is the inflammation of arterial vessels, especially the aorta and its large branches, mainly extracranial branches of the carotid artery , i.e. those that bring blood to the head.

Inflammation most often affects the temporal artery (hence the name of the disease). Apart from them, the disease process may also develop in the ophthalmic artery, posterior ciliary artery and the proximal part of the vertebral artery. Inflammation of the vessels leads to their narrowing and, consequently, to ischemia of the organs to which blood was supplied.

Giant cell arteritis belongs to the group of systemic connective tissue diseases (rheumatic diseases). Research shows that in Europe the incidence is higher in the north of the continent than in the Mediterranean countries. In Scandinavia, 20 per million people suffer from Horton's disease, while in the south of Europe it is only 12. People over 50 suffer mainly from the disease (most cases occur in the 7th decade of life), with a two-fold prevalence of women.

Horton's disease should not be confused with Horton's syndrome, which is a cluster headache.

Giant cell arteritis - causes

The causes of Horton's disease are unknown. It is assumed to be the result of an immune reaction to infectious agents (viral, bacterial) in genetically predisposed individuals. Ie. these factors cause the arterial vessels to be attacked by the immune system.

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Giant cell arteritis - symptoms

Initially, the patient develops systemic flu-like symptoms, i.e .:fever or low-grade fever, anorexia, weight loss, malaise, weakness, chills and sweating, depression. Then, as a result of ischemia in the vascular area, the patient may complain of:

  • chronic headaches, which are usually located in the temporal region (then the temporal artery swells painfully, becoming visible in the form of a tortuous, stringy bulge), and the occipital, parietal and frontal areas. This pain usually occurs at night and disturbs sleep;
  • scalp pains (superficial headaches, e.g. when brushing);
  • claudication in the jaw and tongue (numbness when chewing).

In 1/4 of patients, the arteries supplying blood to the eyes are affected, which is manifested by visual disturbances, such as:

  • double vision
  • visual acuity disorders

Giant cell arteritis often coexists with rheumatic polymyalgia, characterized by pain and morning stiffness in the shoulder and hip girdles. According to research, 50 percent. patients with Horton's disease have polymyalgia rheumatic disease, and 15-25 percent. patients with rheumatic polymyalgia suffer from this type of arteritis.

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Important

Horton's disease can lead to blindness

Vision disorders affect 20-30 percent. patients, about half of whom experience permanent vision loss (usually one-eye). Loss of vision most often occurs suddenly, within a few days, and may be preceded by symptoms such as headache, fever, general symptoms, and jaw claudication.

The most common (91%) cause of this condition is anteriorischemic optic neuropathy , less often posterior neuropathy and obstruction of the middle retinal artery. Their consequence may be the most serious complication of the disease, i.e. permanent vision loss, often preceded by temporary amblyopia, double vision or eye pain. The prognosis is poor as the loss of visual acuity is permanent, although early administration of corticosteroids may improve vision.

Giant cell arteritis is also associated with an increased risk of aortic aneurysm formation, which is usually a late complication of the disease and can dissect, leading to death. In very rare cases, there is a stroke, most often ischemic, and even more rarely there is subarachnoid or intracranial bleeding.

Giant cell arteritis - diagnosis

According to the American Rheumatological Society toThe criteria for the diagnosis of giant cell arteritis include:

  1. Age of the patient - 50 years and above.
  2. New, localized headache develops.
  3. Tenderness of the temporal artery or weakening of the pulse of the temporal artery. 4. OB ≥ 50 mm / h 5. Abnormal temporal artery biopsy.

If 3 out of 5 criteria are met, the disease can be diagnosed. However, the final diagnosis is made on the basis of imaging tests, such as ultrasound, MRI (magnetic resonance imaging) and PET (positron emission computed tomography), which reveal the features of inflammation of the vessel wall. In contrast, ultrasound, angio-CT and arteriography are used to visualize complications such as stenosis / obstruction of the involved arteries, aneurysms, and aortic dissection. Additional tests (ESR, C-reactive protein) and a temporal artery biopsy are helpful in establishing the diagnosis of Horton's disease.

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Giant cell arteritis - treatment

Patients are given glucocorticosteroids. The starting dose is 40-60 mg of prednisone daily in one or two doses. In the case of ocular symptoms, the doses of the drug are higher or pulses of methylprednisolone are used. Prednisone doses are reduced gradually. Treatment is carried out for 2-4 years, but sometimes the therapy with low doses lasts longer.

The hallmark of giant cell arteritis is a sudden improvement after 24-72 hours of glucocorticoid treatment.

Probe

Category:

Cardiology