Arteritis cranialis is an age-related inflammatory disease of the blood vessels that often affects the large and medium-sized arteries of the head. Arteritis cranialis has a massive effect on vision if left untreated.
What is cranial arteritis?
Arteritis cranialis is an inflammation of the walls of the arteries. Women are affected much more often than men, and people of Scandinavian origin are affected more often than other populations. The age of onset of the disease increases significantly after the age of 50. In most cases, the arteries of the head are affected, especially those of the temples, which is why the name arteritis temporalis is sometimes used. Other names for cranial arteritis are Horton’s disease, temporal arteritis, giant cell arteritis, or Horton-Magath-Brown syndrome. Timely treatment of cranial arteritis is critical to prevent permanent tissue damage and vision loss.
Causes
The causes of cranial arteritis have not yet been clearly established. Recent molecular biology research demonstrates that T cells (helper cells) in the vessel wall recognize antigens and send a signal to so-called macrophages (phagocytes) to destroy these putative pathogens. Cranial arteritis is therefore also called an autoimmune disease, which is of genetic origin. The activation of the phagocytes triggers inflammatory tissue destruction, causing the affected arteries to swell and become increasingly poorly perfused. Patients at risk for cranial arteritis include people with polymyalgia rheumatica, systemic lupus erythematosus, rheumatoid arthritis, and severe infections.
Symptoms, complaints, and signs
In most cases, the complaints and symptoms of cranial arteritis are relatively clear, so they point directly to the disease and thus also allow early treatment. Those affected primarily suffer from very severe pain in the area of the head. These headaches are stabbing and can also spread to the neighboring regions. Particularly in the ears or also at the eyes it comes thereby to strong pain. Furthermore, those affected by cranial arteritis also suffer from severe visual disturbances and, in severe cases, blindness. The quality of life of the affected person is significantly limited and reduced by the disease. Weight loss may also occur and may be accompanied by fever or general fatigue and exhaustion. Frequently, cranial arteritis also leads to severe pain in the muscles and thus to restrictions in the movement and everyday life of the affected person. Most patients also suffer from double vision or severe sensitivity of the temples. The patient’s immune system is also weakened by cranial arteritis, so that the patient generally becomes more susceptible to various diseases. Psychological complaints may also occur as a result of the symptoms.
Diagnosis and course
The symptoms of cranial arteritis can vary widely. Sometimes the onset of the disease is characterized by muscle stiffness and pain (myalgia) in the shoulders and hips, fever, and fatigue, as in influenza. However, the most common symptoms of cranial arteritis are often severe headaches and tenderness – usually in both temples. Pain on chewing in the jaw and tongue is also symptomatic. In addition, there is tenderness in the hair and scalp and characteristic loss of vision or seeing double images. If left untreated, cranial arteritis can lead to complications such as permanent blindness, aneurysm, or in isolated cases, stroke. Cranial arteritis is difficult to diagnose, so other diseases must first be ruled out. A diagnosis is made based on a comprehensive history with physical examination and on laboratory tests. These check liver values, erythrocyte sedimentation rate, and levels of certain inflammatory markers. The standard for diagnosing cranial arteritis is a biopsy; imaging techniques such as magnetic resonance angiography, ultrasound, or positron emission tomography may also be used to supplement this.
Complications
Significant visual impairment occurs as a result of cranial arteritis.In the worst case, the patient can lose all vision and go blind. Women are more often affected by cranial arteritis than men and consequently suffer more often from the complications of the disease. Most often there is a stabbing pain in the head, which comes with visual disturbances or complete blindness. The affected person also suffers from fever and fatigue and a general feeling of illness. The muscles and extremities also ache, reducing the quality of life tremendously. Before blindness, veil vision and double vision occur. It is not uncommon for patients to suffer a stroke, and in the worst case this can lead to death. The treatment is carried out with the help of medication. In most cases, the symptoms improve after a few days, but the entire treatment can take several years. In most cases, eye complaints and high blood pressure are also treated. In many cases, the therapy causes weight gain and a weakening of the immune system. As a result, the patient gets sick more often with flu and other infections.
When should you see a doctor?
Cranial arteritis must always be treated by a doctor. If treatment is not received, the disease can have a negative effect on the patient’s vision, leading in the worst case to complete blindness. For this reason, urgent treatment by a physician is necessary. As a rule, a doctor should be consulted if there are sudden visual disturbances associated with severe headaches. Likewise, thick arteries at the temples may be a sign of cranial arteritis and should be examined by a medical professional. Furthermore, a physician should also be consulted if the patient develops a fever or is fatigued and tired. It is not uncommon for cranial arteritis to lead to muscle pain or significant weight loss. Without treatment, this can also lead to a stroke, which can be fatal for the affected person. Usually, cranial arteritis can be diagnosed by a general practitioner. Further treatment takes place with the help of medication and the symptoms should be relatively well limited.
Treatment and therapy
If cranial arteritis can be diagnosed and therefore treated early, the prognosis is usually very good. Arteritis cranialis is treated with high doses (1 mg/kg/day) of corticosteroid medications such as prednisone. Since immediate treatment is sought, drug treatment can begin before the diagnosis is confirmed. Although improvement may occur after only a few days, prolonged use of the medication is required for one to two years or longer. After the first few weeks, the dose may be gradually decreased until only a low dose of corticosteroids is needed to control inflammation. Some of the symptoms may return briefly during treatment. Corticosteroids are powerful anti-inflammatory drugs. Therefore, especially at high doses, long-term use can cause serious side effects. These include osteoporosis, high blood pressure, muscle weakness, glaucoma, or cataracts. Other possible side effects of corticosteroid therapy for cranial arteritis include weight gain, elevated blood sugar levels, and a weakened immune system. Calcium and vitamin D supplements or other medications are used to treat the side effects.
Outlook and prognosis
Cranial arteritis has a good prognosis if diagnosed early and treatment is started quickly. With drug treatment, there is a possibility of relief of symptoms. For a good and stable healing picture, the treatment must be carried out over several years. However, already after a few days or weeks a clear improvement is noticeable. If the medication is discontinued independently and too early, the disease relapses and the symptoms reappear. Without medical care, the impairments continuously increase in extent and new symptoms appear. The progressive course of the disease can lead to blindness of the patient in unfavorable circumstances. In addition, the occurrence of a stroke is possible in individual cases.This usually leads to lifelong impairments, paralysis and functional disorders. In addition, it can have a fatal course if treatment is delayed or without intensive medical care. The difficulty with cranial arteritis is diagnosis. The disease is easily confused with other diseases because of the symptoms. This leads to a delay in medical consultation or initial misdiagnosis at the doctor’s office. The delay in the onset of the disease triggers a continuous deterioration of vision. Once correction is made and treatment begins, the symptoms gradually resolve.
Prevention
Prevention of cranial arteritis is not possible. However, coping with the side effects of the medications is a major challenge. A healthy diet of fresh fruits and vegetables, whole grains, lean meats and fish, and limiting salt, sugar, and alcohol is the best foundation for countering potential problems. Regular exercise for at least 30 minutes a day strengthens the immune system and well-being to get along tolerably with treatment for cranial arteritis.
Follow-up
Options for aftercare usually prove relatively difficult with cranial arteritis. Affected individuals are dependent on a medical condition for this disease in most cases. In the treatment of the disease, the patient usually has to take medicines. Care should always be taken to ensure that the medication is taken regularly, and interactions with other medications should also be considered. In most cases, patients are dependent on prolonged treatment. Since the treatment of cranial arteritis can also cause serious side effects, the affected person should have regular check-ups. A healthy lifestyle with a healthy diet also has a very positive effect on the further course of cranial arteritis. Weight gain should be avoided at all costs. Care by friends and relatives can also have a very positive effect on the course of the disease and prevent further complaints. Through contact with other sufferers of cranial arteritis, there can be an exchange of information, which can also have a positive effect on the further course of the disease.
What you can do yourself
Giant cell arteritis is a very serious rheumatic vascular disease that requires mandatory treatment by a physician. Affected individuals should absolutely follow a doctor-prescribed treatment with cortisone consistently, even if it results in undesirable side effects. However, patients can also alleviate the latter by taking self-help measures. Since the administration of cortisone increases the risk of osteoporosis, calcium should be taken at the same time and attention paid to sufficient sunlight or vitamin D should be substituted. Potential vascular occlusion can be prevented by taking acetylsalicylic acid (ASA), which is contained in many over-the-counter pain relievers. Since ASA attacks the stomach lining, it should be protected by a proton inhibitor. Naturopathic methods can also be used to support the treatment. A classic in the therapy of rheumatic complaints is birch, which is used as juice or tea. A decoction of birch leaves can also be applied directly to painful areas of the body. Nettle is also said to have a positive effect on inflammatory processes in the body. Willow bark is a natural source of ASA and is drunk as a tea, although it tastes very peculiar. Meanwhile, however, willow bark-based tablets are also available. Often, moving the jaw can exacerbate the severe headache almost always associated with giant cell arteritis. In acute attacks, therefore, sufferers should only eat soft or liquid food and under no circumstances should they chew vigorously.
