(Temporal arteritis, cranial arteritis; Horton’s disease)
Giant cell arteritis primarily affects the aorta, major arteries leading from the aorta in the neck, and extracranial carotid branches of. The simultaneous occurrence of PMR is frequent. Symptoms may include headache, blurred vision, a painful temporal artery and pain in the jaw muscle. Fever, weight loss, malaise and fatigue are also common. ESR and C-reactive protein are usually increased. The diagnosis is made clinically and confirmed by a biopsy of the temporal artery. Treatment with high-dose corticosteroids and aspirin is very effective and prevents vision loss.
The giant is a relatively common form of vasculitis in the US and Europe. The incidence varies by ethnicity. Autopsy studies suggest that the disease may be more common than clinical signs. Women are affected more often. The mean age of onset is about 70 years, with a fluctuation range of 50 to> 90 years. Around 40-60% of patients with giant cell arteritis have polymyalgia rheumatica. The intracranial vessels are not usually affected.
Giant cell arteritis primarily affects the aorta, major arteries leading from the aorta in the neck, and extracranial carotid branches of. The simultaneous occurrence of PMR is frequent. Symptoms may include headache, blurred vision, a painful temporal artery and pain in the jaw muscle. Fever, weight loss, malaise and fatigue are also common. ESR and C-reactive protein are usually increased. The diagnosis is made clinically and confirmed by a biopsy of the temporal artery. Treatment with high-dose corticosteroids and aspirin is very effective and prevents vision loss. The giant is a relatively common form of vasculitis in the US and Europe. The incidence varies by ethnicity. Autopsy studies suggest that the disease may be more common than clinical signs. Women are affected more often. The mean age of onset is about 70 years, with a fluctuation range of 50 to> 90 years. Around 40-60% of patients with giant cell arteritis have polymyalgia rheumatica. The intracranial vessels are not usually affected. Pathophysiology vasculitis may be localized, multifocal or widespread occur. Primarily are arteries with elastic tissue, most often temporal, cranial or other arteries of the carotid affected by the disease. Branches in the aortic arch, coronary and peripheral arteries may also be affected. Mononuclear cell infiltrates in the adventitia form granulomas with activated T-cells and macrophages. form giant cells, if present, cluster near the destroyed elastic lamina. The intima is significantly thickened with concentric constriction and occlusion of the lumen. Symptoms and discomfort symptoms can gradually use over several weeks or abrupt. Patients present with systemic symptoms such as fever (usually geringgradig), fatigue, malaise, unexplained weight loss and sweating. In some patients, fever of unknown origin is first diagnosed. After all, most patients develop symptoms associated with the affected arteries. Severe, sometimes raging headache (temporal, occipital, frontal or diffuse) are the most common symptom. They are accompanied by pain of the scalp, triggered by touching the scalp or combing hair. The visual disturbance include diplopia, scotoma, ptosis, blurred vision, and loss of vision (which is an ominous sign). In the short periods of partial or complete loss of vision (amaurosis fugax) in one eye quickly follows the permanent irreversible loss of vision. If left untreated, the other eye can also be affected. However, a complete bilateral blindness is unusual. Vision loss is caused by eien arteritis of the branches of the ophthalmic artery or posterior ciliary arteries, leading to ischemia of the optic nerve. The Funduscopic findings may include ischemic neuritis with pallor and edema of the optic disc, scattered cotton-like patches and small hemorrhages. Later the optic nerve atrophy. Rarely results in a central blindness by an infarction in the visual cortex, induced by arterial lesions in the distal neck region or the base of the brain. The incidence of visual impairment has declined over the past five decades, and the recovery rates have improved, probably because the temporal arteritis is detected and treated before they develop vision problems. Intermittent claudication (ischemic muscle pain) is evident in the jaw muscles and muscles of the tongue or the extremities. A claudication of the jaw is especially noticed when solid food is chewed. The restriction of the jaw opening is associated with an increased risk of visual symptoms. Neurological manifestations such as stroke and transient ischemic attacks may result if the carotid or vertebrobasilar arteries are narrowed or closed. Thoracic aortic aneurysms and aortic dissection are serious, often late complications can occur without signs of other symptoms. Diagnosis ESR, C-reactive protein and total blood count biopsy, usually temporal artery Suspected giant is> 55 years with patients when one of the following symptoms develop, especially when they show symptoms of systemic inflammation: a new way headaches each new symptom or sign of an artery ischemia above the neck jaw pain when chewing tenderness temporal artery unexplained subacute fever or anemia the diagnosis is more likely if patients also have symptoms of polymyalgia rheumatica. The physical examination can determine swelling and tenderness with or without knotting or redness over the temporal arteries. The temporal arteries may appear as pronounced. A temporal artery, which rolls beneath the fingers of the examiner instead of collapsing, is abnormal. The major arteries of the neck and limbs and the aorta should be evaluated for vascular sounds. Suspicion of AT ESR, CRP and blood are determined. In most patients, ESR and CRP are elevated; in chronic disease anemia is common. Occasionally, the platelet count is increased, whereas serum albumin and total protein are low when they are measured. A slight leukocytosis is often stated, but is not specific. If the diagnosis is suspected giant cell arteritis, the biopsy of an artery is recommended. Since inflamed segments often alternate with normal segments, if possible, a segment that appears abnormal, be analyzed. Normally, the temporal artery is biopsied from the side, which is symptomatic, but it can be biopsied and the occipital artery when it appears abnormal. The optimal length for the removal of the temporal artery is unclear, but up to 5 cm long samples increase the accuracy. The additional diagnostic value of a contralateral biopsy is low. Therapy should not await the implementation of the biopsy. Since inflammation slowly dies, the temporary artery biopsy can be performed within 2 weeks after the treatment was started. Imaging of the aorta and its branches should be done at the time of diagnosis and then at regular intervals thereafter, even if no suggestive symptoms or signs are present (see table: Imaging techniques in Takayasu arteritis). Therapy Corticosteroids Low-dose aspirin therapy should be started immediately when a giant is suspected, even if the biopsy may not take place until several days later. Tips and risks when patients show> 55 years emerging headache, jaw claudication, sudden vision changes, and / or tenderness of the temporal arteries, immediate treatment should be considered with corticosteroids against giant. The cornerstone of treatment is corticosteroids. These quickly reduce symptoms and prevent vision loss in most patients. The optimal dose, Ausschleichzeitplan and total duration of treatment are under discussion. In most patients, an initial dose of 40-60 mg of prednisone is administered p.o. once a day (or equivalent) for 4 weeks, followed by gradual tapering off effectively. If the patients have vision problems, an attempt at a dose of 500-1000 mg methylprednisolone may i.v. be taken once a day for 3-5 days to prevent a further decline of vision, particularly in the contralateral eye. Preserving the vision is probably more depend on how fast is started with the use of corticosteroids, as their dose. An optic nerve infarction, once started, can not be reversed, regardless of the corticosteroid. If symptoms moderating, prednisone can gradually, depending on the response of the patient, be tapered from up to 60 mg / day at the following doses: 5-10 mg / day every week at 40 mg / day, 2-5 mg / day every week to 10-20 mg / day, then to 1 mg / day in the following month, until the drug is discontinued. BSG alone should not be used (and the disease activity) to assess the patient’s response. For example, other factors, such as monoclonal gammopathy ESR can increase in the elderly. The clinical symptoms have to be considered. C-reactive protein can sometimes be useful as ESR. Most patients require a minimum of 2 years of treatment with corticosteroids. The long-term use of corticosteroids can have serious side effects and should therefore be limited if possible. More than half of patients taking these drugs is drug-related complications. Therefore, alternative therapies are being researched. If the patient can not tolerate corticosteroids or if symptoms return when the dose is tapered off, methotrexate (0.3 mg / kg / week) useful. In some patients methotrexate can help save corticosteroids and to reduce the number of relapses. Older patients with Dauerkortikosteroidtherapie should receive bisphosphonates for prevention of osteoporosis. In a systematic review of the evidence of biological agents was weak proven. In a randomized controlled trial, the anti-TNF drug infliximab had no benefit and possible damage was detected. Low-dose aspirin (81-100 mg po once daily) prevents ischemic events and should be prescribed to all patients, unless it is contraindicated. Summary The giant is non vasculitis of the great arteries, which affects the aorta and its branches in the head and neck region. Many patients show a rheumatica polymyalgia. Symptoms include headache, jaw claudication, pain on palpation of the temporal arteries and general symptoms. Blood count, ESR and CRP are determined, and there is a biopsy of the temporal artery. The treatment consists (with immediate onset) and low-dose aspirin in the administration of corticosteroids.