Brief overview
- Symptoms: New onset of severe headache at one temple, especially when chewing or turning the head, visual disturbances, nonspecific symptoms such as fever and fatigue.
- Treatment: Cortisone preparations, other drugs against side effects, if necessary additional anti-inflammatory antibody preparations
- Causes and risk factors:Autoimmune disease, probably favored by genetic factors and triggered by environmental factors, exact causes are still unclear; possible risk factors are infections such as chickenpox or rubella
- Diagnosis:Based on symptoms; ultrasound, magnetic resonance imaging or positron emission tomography of the arteries; sampling and microscopic examination of a temporal artery
- Prognosis:Without therapy, about one-third of those affected go blind; if diagnosed early, symptoms usually disappear; rarely relapses; in some cases, those affected take medication permanently; rarely chronic course
- Prevention:No general prevention known, regular control examinations to prevent possible relapses
What is temporal arteritis?
Sometimes arteritis temporalis is referred to as giant cell arteritis. Strictly speaking, however, arteritis temporalis is a symptom of giant cell arteritis. In the course of this vasculitis, other vessels outside the temporal region also become inflamed. Arteriitis temporalis also occurs in other inflammatory diseases.
The exact distinction between arteritis temporalis and giant cell arteritis is unclear to date. Experts suspect that they are different stages of the same disease.
What is giant cell arteritis?
In this vasculitis, large and medium-sized vessels are affected. Most commonly, the disease occurs in vessel branches of the carotid artery. These vessels supply blood to the temporal region, the back of the head, and the eyes. In some patients, giant cell arteritis – also called RZA disease – affects the aorta or larger vessels in the trunk and extremities. Coronary vessels are also sometimes affected (coronaritis).
The disease causes cells in the vessel wall to proliferate and eventually constrict the affected vessel. As a result, the blood supply is often insufficient, especially during physical exertion. Depending on the organ affected, this causes corresponding symptoms.
Frequency
Giant cell arteritis is one of the most common rheumatic vascular diseases and the most common vasculitis. It is usually manifested by arteritis temporalis. The risk of disease increases with age. Women are affected by giant cell arteritis significantly more often than men. About half of those with the disease have polymyalgia (polymyalgia rheumatica). The distinction between temporal arteritis or giant cell arteritis and polymyalgia is often difficult.
In polymyalgia rheumatica, large arteries are also inflamed, especially the subclavian artery. Doctors assume that polymyalgia rheumatica is a mild form of giant cell arteritis, but primarily affects joints and tendons. As a result, affected individuals usually complain of severe shoulder and upper arm pain and often pelvic distress.
What are the symptoms of temporal arteritis?
Almost all patients with temporal arteritis have particularly severe headaches. Most have general symptoms of the disease long before the first headache.
More than 70 percent of people with temporal arteritis suffer from new onset, severe headaches. These are most often described as drilling to stabbing and usually occur on one side of the temple. The pain intensifies when sufferers chew, cough, or turn their head.
When sufferers chew solid foods, the masseter muscle becomes more stressed and requires more nutrients and oxygen. If the supply is not guaranteed in the case of a damaged artery, pain occurs in the area of the temple, the scalp or a painless feeling of lockjaw (claudication masticatoria). In some cases, affected persons have to pause during a meal as a result.
Visual disturbances in giant cell arteritis of the ocular vessels
If inflamed vessels are present in the eye in addition to or instead of arteritis temporalis, both the optic nerve and eye muscles function only to a limited extent. Just like the muscles, the optic nerve must be constantly supplied with blood. If the supplying arteries change pathologically, visual disturbances usually occur. These include fleeting loss of vision (amaurosis fugax), in which those affected suddenly see nothing in one eye.
If giant cell arteritis affects eye vessels, it is a medical emergency: permanent blindness is imminent.
Other symptoms of temporal arteritis and giant cell arteritis
Even some time before the typical headaches of temporal arteritis appear, those affected often suffer from non-specific symptoms of the disease. They feel fatigued or repeatedly have a slightly elevated body temperature. If only the aorta is affected in giant cell arteritis, fever may be the only symptom of the disease. In addition, lack of appetite and weight loss are among the accompanying symptoms of giant cell arteritis.
In addition to arteritis temporalis or inflammation of the ocular vessels, the following symptoms are common in giant cell arteritis:
- Central neurological deficits: If vessels inside the brain are affected by giant cell arteritis – for example, if brain regions are not sufficiently supplied with oxygen and nutrients – a stroke with corresponding symptoms such as paralysis, speech disorders or dizziness is a possible consequence.
- Blood pressure differences and arm pain: If the aorta is affected, it often becomes apparent that the blood pressure differs between the two arms. In addition, a palpable pulse at the wrist disappears in some sufferers. Others suffer from pain in the arms that occurs mainly during exertion (arm claudication).
- Aneurysm and dissection: If a section of the aorta in the thorax is affected, bulges (aneurysm) and vessel tears (dissection) occur more frequently and may be life-threatening.
- Angina pectoris: If giant cell arteritis affects the coronary arteries and triggers coronary inflammation, sufferers experience symptoms similar to those of a heart attack. These include, for example, a feeling of pressure and pain in the chest, a kind of trepidation, palpitations, shortness of breath, sweating or dizziness.
In about 20 percent of cases, arteritis temporalis occurs in the context of polymyalgia rheumatica. Conversely, about 30 to 70 percent of patients with giant cell arteritis develop polymyalgia. Those affected then suffer from additional pain in the shoulder, pelvic area or neck muscles.
How is giant cell arteritis treated?
After the diagnosis of temporal arteritis, doctors advise immediate use of a cortisone preparation. For the first four weeks, doctors recommend a dosage of one milligram of prednisolone per kilogram of body weight. If the symptoms disappear as a result of the therapy and the inflammation values in the blood normalize, the person treating the patient usually reduces the dose continuously. If symptoms reappear, the physician gives more prednisolone again.
The attending physician works out a precise intake regimen for this arteritis temporalis therapy together with his patient. If blindness is imminent, prednisolone therapy is given for three to five days in high doses via the vein.
The guidelines of the German Society of Neurology recommend 60 to 100 milligrams of a cortisone preparation for arteritis temporalis if the eyes are not involved. For unilateral blindness that has just occurred, 200 to 500 milligrams, and if blindness is imminent, a high dose of 500 to 1000 milligrams.
If experts previously recommended the preventive use of the “blood thinner” ASA (acetylsalicylic acid), the hoped-for prophylactic effect has not been confirmed.
With a so-called maintenance therapy, a life with giant cell arteritis without further symptoms is quite possible. The therapy is continued for several years with a lower dose of the cortisone preparation and the supplementary drugs. In half of the cases, the therapy ends after about two years.
Cytostatic drugs or immunosuppressants
Cell growth inhibitors (cytostatics) or drugs that suppress the immune system (immunosuppressants) are possible agents that the doctor gives in some cases to supplement cortisone therapy. Such agents include methotrexate, which is also used in cancer therapy, or azathioprine as an immunosuppressant.
New form of therapy with tocilizumab
A new approach in the therapy of giant cell arteritis is a so-called “monoclonal antibody”. This is used as a drug under the name tocilizumab. The antibody is directed against the receptor of the immune messenger interleukin-6 (IL-6). This increases inflammation. The administration of tocilizumab reduces inflammatory diseases such as giant cell arteritis. Doctors give this active ingredient as a supplement to cortisone preparations and at the same time reduce the dose of cortisone.
How long such therapy must be maintained varies from patient to patient. In mild cases, therapy is terminated after a few years in about half of the cases without relapse. In other cases, sufferers take permanent medication for a lifetime of giant cell arteritis.
Causes and risk factors
Arteritis temporalis, or giant cell arteritis, is a rheumatic disease in which the immune system functions incorrectly. Certain immune cells, called T cells, cause an autoimmune reaction. Why this happens has not yet been adequately researched. It is possible that the disease is triggered by infections with viruses (chickenpox, ringworm) or bacteria (Mycoplasma pneumoniae, chlamydia).
Since not all people with such infections develop arteritis temporalis, there is probably a genetic susceptibility. People with certain proteins on white blood cells (HLA-DR4) are more likely to develop the disease. In addition, arteritis temporalis is seen more often in people with polymyalgia, another rheumatic pain disorder.
Examinations and diagnosis
First, the physician conducts an initial interview (anamnesis). If the suspected disease is confirmed, imaging and tissue sampling follow. In some cases, the blood values in a blood test show elevated levels of inflammation. If at least three of the following five criteria apply to an affected person, there is a probability of more than 90 percent that the patient has arteritis temporalis:
- Age over 50 years
- First or new onset of headache
- Altered temporal arteries (pressure painful, weakened pulse)
- Increased sedimentation rate (blood test)
- Tissue changes of a temporal artery
Further examinations
In most cases, the physician will perform an ultrasound examination of the temporal arteries to visualize the blood flow (Doppler sonography). The temporal artery can also be assessed with magnetic resonance imaging (MRI). To do this, the doctor first injects a specific contrast agent into a vein before moving the patient’s head into the MRI tube on a movable couch. This may reveal vascular changes in other arteries that sometimes occur in giant cell arteritis.
Tissue sampling for temporal arteritis
If signs of the disease and imaging examinations point to temporal arteritis, in many cases the physician takes a tissue sample (biopsy) from the affected temporal region and examines it microscopically. Since the disease cannot be detected in an ultrasound examination in every patient, it is considered safer to take a tissue sample even if the ultrasound result is unremarkable. In some cases, an additional sample is taken from the other side of the temple.
A biopsy of the temporal artery is considered the gold standard for diagnosing temporal arteritis.
Prior to the biopsy, the physician carefully selects the site for sample collection. He also makes sure that the piece of vessel taken is sufficiently long (around one centimeter). This is because the inflammatory vascular changes with giant cells that are typical of giant cell arteritis only occur in sections of the vessel walls. The wall areas in between look normal.
Course of the disease and prognosis
Without therapy, approximately 30 percent of those affected go blind. However, with early diagnosis and subsequent therapy, the symptoms disappear permanently in almost all patients. Only rarely does giant cell arteritis recur or, for example, turn into chronic temporal arteritis.
Prevention
Anyone who has already had such a disease and has been successfully treated should go for regular check-ups for prevention and early detection of possible relapses.
