
All iLive content is medically reviewed or fact checked to ensure as much factual accuracy as possible.
We have strict sourcing guidelines and only link to reputable media sites, academic research institutions and, whenever possible, medically peer reviewed studies. Note that the numbers in parentheses ([1], [2], etc.) are clickable links to these studies.
If you feel that any of our content is inaccurate, out-of-date, or otherwise questionable, please select it and press Ctrl + Enter.
Gigantocellular arteritis
Medical expert of the article
Last reviewed: 04.07.2025
Giant cell arteritis is a granulomatous inflammation of the aorta and its main branches, primarily the extracranial branches of the carotid artery, with frequent involvement of the temporal artery. The disease usually occurs in patients over 50 years of age and is often combined with rheumatic polymyalgia.
Epidemiology
Giant cell arteritis affects mainly people of the Caucasian race. The incidence varies widely from 0.5 to 23.3 cases of the disease per 100 thousand people over 50 years of age. Its increase is noted in older age groups. Women are slightly more often affected than men (ratio 3:1). The disease is more common in Northern Europe and America (especially among Scandinavian immigrants) than in the southern regions of the globe.
How does giant cell arteritis manifest itself?
Sometimes giant cell arteritis begins acutely, patients can clearly note the day and hour of the onset of the disease, but in most cases the symptoms of the disease appear gradually.
Constitutional signs of the disease include fever (subfebrile and febrile), which is often the only symptom of the disease for a long time, profuse sweating, general weakness, anorexia, weight loss (up to 10 kg or more over several months), and depression.
Vascular disorders depend on the localization of the process to the arterial bed. When the temporal artery is affected, there is a constant, acutely developing intense bilateral headache localized in the frontal and parietal region, pain when touching the scalp, swelling, edema of the temporal arteries and weakening of their pulsation. Headache when the occipital artery is affected is localized in the occipital region.
Pathology of the maxillary artery leads to "intermittent claudication" of the masticatory muscles, causeless toothache or "intermittent claudication" of the tongue (if the process is localized in the lingual artery). Changes in the external carotid artery lead to facial edema, impaired swallowing and hearing
Inflammation of the arteries supplying the eyes and eye muscles can lead to visual impairment, often irreversible, which is the first symptom of the disease. Ischemic choriorentinitis, corneal edema, iritis, conjunctivitis, episcleritis, scleritis, and anterior ischemic optic neuropathy have been described. Transient visual impairment (amavrosis fugax) and diplopia are very common. The development of blindness is the most serious early complication of giant cell arteritis.
Pathological changes (aneurysm) of the aorta (mainly the thoracic region) and large arteries occur, as well as symptoms of tissue ischemia.
Rheumatic polymyalgia occurs in 40-60% of patients, and in 5-50%, signs of an inflammatory process are detected during a biopsy of the temporal artery.
Joint damage occurs in the form of symmetrical seronegative polyarthritis, reminiscent of rheumatoid arthritis in the elderly (involving mainly the knee, wrist and ankle joints, less often the proximal interphalangeal and metatarsophalangeal joints), or mono-oligoarthritis.
Various symptoms of upper respiratory tract pathology occur in 10% of patients and may be the first signs of the disease. Unproductive cough against the background of fever predominates. Much less common are pains in the chest and throat. The latter are often significantly pronounced and are apparently caused by damage to the branches of the external carotid artery, mainly a. рharyngea ascendens. Inflammatory changes rarely affect the lungs directly. There are only isolated descriptions of such cases.
How to recognize giant cell arteritis?
The most important laboratory signs reflecting giant cell arteritis are a marked increase in ESR and CRP. However, some patients have normal ESR values. A more sensitive indicator of activity is an increase in the concentration of CRP and IL-6 (more than 6 pg/ml).
High-resolution ultrasound does not differentiate inflammatory vascular lesions from atherosclerotic ones. MRI can detect inflammatory changes in the thoracic aorta, the formation of an aneurysm.
Radiography and CT of the lungs reveal basal interstitial fibrosis, diffuse reticular changes in the pulmonary pattern, multiple nodules, and aneurysms of the thoracic aorta. Results of bronchoalveolar lavage studies in patients with clinical signs of respiratory pathology but without radiological changes reveal signs of T-lymphocyte alveolitis with a predominance of CD4+ lymphocytes.
Diagnosis of giant cell arteritis is based on the classification criteria of the ARA. The disease should be suspected in all patients over 50 years of age with severe headaches, visual impairment, symptoms of rheumatic polymyalgia, a significant increase in ESR and anemia. To confirm the diagnosis, it is advisable to perform a biopsy of the temporal artery. However, since focal segmental vascular lesions often develop in giant cell arteritis, negative biopsy results do not allow this diagnosis to be completely excluded. In addition, this does not serve as a basis for not prescribing glucocorticosteroids.
Differential diagnostics
Differential diagnostics of giant cell arteritis is carried out with a wide range of diseases that occur with symptoms of rheumatic polymyalgia and damage to large vessels. These include rheumatoid arthritis and other inflammatory diseases of the joints in the elderly, damage to the shoulder joint (scapulohumeral periarthritis), inflammatory myopathies. malignant neoplasms, infections, hypothyroidism (autoimmune thyroiditis), Parkinson's disease, systemic amyloidosis. atherosclerotic vascular lesions.
Who to contact?
How to treat giant cell arteritis?
If giant cell arteritis is suspected and other diseases (tumors, etc.) have been excluded, treatment with glucocorticosteroids should be started immediately to prevent the development of blindness and irreversible damage to internal organs.
Glucocorticosteroids are the main method of treatment for giant cell arteritis. Prednisolone is used at a dose of 40-60 mg / day in several doses until the ESR is normalized and symptoms disappear. Reduce the dose by 2.1 s mg / day every 2 weeks until reaching 20 mg / day, then by 10% every 2 weeks to 10 mg / day, then 1 mg every 4 weeks 1 week if there are no visual impairment or damage to large vessels, an adequate starting dose of prednisolone can be less than 20 mg / day. In the process of reducing the dose of prednisolone, carefully monitor the dynamics of symptoms, monitor ESR every 4 weeks during the first three months, then every 12 weeks for 12-18 months after completion of treatment.
In severe cases of giant cell arteritis, the dose of glucocorticoids should be increased to 60-80 mg/day, or pulse therapy with methylprednisolone should be performed followed by a transition to a maintenance dose of prednisolone 20-30 mg/day, or methotrexate (15-17.5 mg/week) should be added to the treatment. When prescribing methotrexate, it is necessary to remember the possibility of pneumonitis developing against its background.
The duration of therapy is assessed individually for each patient. If there are no clinical symptoms of the disease during 6 months of taking prednisolone at a dose of 2.5 mg/day, treatment can be stopped. Taking acetylsalicylic acid at a dose of 100 mg/day reduces the risk of blindness and cerebrovascular accidents.
What is the prognosis for giant cell arteritis?
In general, the prognosis for the life of patients with giant cell arteritis is favorable. Five-year survival is almost 100%. However, there is a serious risk of developing various complications of the disease, primarily damage to the arteries of the eyes, leading to partial or complete loss of vision.