What happens to the vessels
Immune cells penetrate the artery wall and form foci of inflammation in it with large multinucleated cells—hence the name “giant cell.” The wall thickens from the inside, the lumen narrows, blood flow slows down, and sometimes the vessel closes with a blood clot. Most often, the superficial temporal, occipital and ophthalmic arteries are affected, but inflammation can involve the aorta and its large branches. It is damage to the posterior ciliary arteries that supply the optic nerve that leads to sudden and permanent loss of vision.
- Large and medium arteries of the head and neck are affected
- Possible involvement of the aorta and subclavian arteries
- The lumen of the vessel narrows, tissues do not receive enough blood
- Optic nerve ischemia causes irreversible blindness
- Some patients also have polymyalgia rheumatica
Causes and risk factors
The exact reason is unknown. It is believed that with a certain hereditary predisposition, the immune system begins to react to its own structures of the vascular wall. Age is the main factor: before the age of 50, the disease is extremely rare, and with each decade after 50, the risk increases. Women get sick more often than men. The provoking role of specific infections is discussed, but has not been convincingly proven. It is not the disease itself that is inherited, but only a predisposition, so familial cases are rare.
- Age over 50
- Female gender
- Genetic predisposition
- History of polymyalgia rheumatica
- European origin - slightly higher frequency
Symptoms and connection with polymyalgia rheumatica
The headache is usually new, different from previous ones, usually one-sided, in the temple or back of the head, worse at night. Many people notice that it hurts to comb their hair or lie on a pillow. A characteristic symptom is “intermittent claudication of the jaw”: while chewing, the muscles become tired and sore, and you have to pause. About half of the patients have common symptoms: low-grade fever, weakness, loss of appetite and weight. In some patients, the disease is combined with polymyalgia rheumatica - morning pain and stiffness in the shoulder and pelvic girdle.
- New headache in the temporal region
- Firm, painful, weakly pulsating temporal artery
- Pain in the masticatory muscles when eating
- Scalp soreness
- Blurred, double, or loss of vision
- Low-grade fever, weakness, weight loss
- Pain and stiffness in the shoulders and hips
Diagnostics
The first thing to look at is inflammatory markers: ESR and C-reactive protein are almost always significantly elevated in active arteritis; a general blood test often shows anemia and increased platelets. Then the vessels themselves are assessed. Ultrasound of the temporal arteries with a high-frequency sensor allows you to see a characteristic thickening of the wall, the so-called halo symptom. The final confirmation is provided by a biopsy of the temporal artery, but the inflammation in the vessel is unevenly distributed, so a negative result does not eliminate the diagnosis. If aortic damage is suspected, CT or MR angiography is performed. An examination by an ophthalmologist with an assessment of the fundus is mandatory.
- ESR and C-reactive protein
- Complete blood count with platelets
- Ultrasound of the temporal and axillary arteries
- Temporal artery biopsy
- CT or MR angiography of the aorta and its branches
- Examination by an ophthalmologist with fundus
Treatment and observation
The basis of treatment is glucocorticoids. If vision is threatened, they are prescribed immediately, even before the biopsy, because the delay is measured in hours. The initial dose is held for several weeks and then slowly reduced over months, based on how you feel and markers of inflammation. To reduce the duration of hormonal therapy and the number of exacerbations, the doctor may add a basic or genetically engineered drug. At the same time, they protect bones and metabolism: they control blood pressure, sugar and bone density, prescribe calcium and vitamin D. You cannot quit hormones on your own - this is a common cause of relapse.
- Glucocorticoids immediately if there is reasonable suspicion
- Slow dose reduction under medical supervision
- Basic or genetically engineered drugs according to indications
- Preventing osteoporosis: calcium and vitamin D
- Monitoring blood pressure, glucose and body weight
- Regular assessment of ESR and C-reactive protein