Last updated:
7/18/2025
Years published: 1986, 1988, 1994, 1998, 2001, 2002, 2007, 2019, 2025
NORD gratefully acknowledges Gioconda Alyea, MD (FMG), MS, National Organization for Rare Disorders, Andy Nguyen, NORD Editorial Intern from the Massachusetts College of Pharmacy and Health Sciences, and Bhaskar Dasgupta, MD, Professor of Medicine, Department of Rheumatology, Southend University Hospital, Westcliff, Essex, UK, for assistance in the preparation of this report.
Giant cell arteritis (GCA), also known as temporal arteritis, is a systemic vasculitis that primarily affects medium to large arteries, particularly in the head and neck. It is the most common blood vessel disorder in people over 50 years old that causes inflammation of medium and large-sized arteries in the body (vasculitis). GCA causes changes in blood vessel walls leading to poor blood circulation. The most affected blood vessels in giant cell arteritis are the temporal artery and other cranial arteries (now called cranial-GCA), but inflammation of the aorta and other large arteries in the body can occur as well and may present differently (now called large vessel-GCA). If left untreated, this can lead to a medical emergency where sudden blindness occurs.
When the temporal and other cranial arteries are affected, the signs and symptoms include arm pain, pulsing headaches on one side or on the back of the head, jaw pain, scalp tenderness, double vision or other visual disturbances and a bulging temporal artery that is tender with skin edema and redness. It can also present with constitutional symptoms such as polymyalgia, fevers, anorexia and weight loss, when the large vessels are affected.
The cause of giant cell arteritis is still unknown but is thought to be from the immune system causing damage to the body’s own blood vessels.
Polymyalgia rheumatica is an inflammatory disorder that is closely related to giant cell arteritis and occurs in 40% to 60% of patients with giant call arteritis. About 15% to 20% of people with polymyalgia rheumatica will have giant cell arteritis. The number of people with giant cell arteritis is estimated to be above 200,000 meaning that it is not strictly considered a rare disease in USA.
The main treatment includes steroids (corticosteroids) that help with symptoms and reoccurrence and medications that weaken the immune system. Other medications have also been approved to treat GCA.
Giant cell arteritis (GCA) is a systemic vasculitis that commonly affects medium and large arteries, particularly the temporal arteries located on either side of the head. More than 60% of patients report headaches, typically new in onset, severe and localized around the temples. The temporal artery may appear thickened or nodular, tender to touch or pulsatile early in the course but may later become non-pulsatile if occluded.
In advanced cases, the artery may bulge visibly, with a twisted or knotted appearance under the skin. This is often accompanied by localized redness and swelling of the scalp. The frontal and parietal branches of the artery may also be visibly distended.
Visual symptoms which may be serious and potentially irreversible:
GCA can affect arteries located beyond the head:
GCA often presents with non-specific, systemic inflammatory signs, including:
The cause of giant cell arteritis is still not fully known but it is thought to be an autoimmune disorder, where the body’s defense system used against invading organisms is used instead to attack normal healthy tissues in the arteries. The immune cells come together at the site where they are attacking the body and form giant cells. These giant cells produce substances that damage the walls of the artery and lead to further inflammation. The inflammation narrows the arteries leading to decreased blood flow in the body organs that these arteries supply.
Studies have shown that variants in genes for human leukocyte antigens (HLA), which are part of the immune response, may predispose a person to have giant cell arteritis. There are other gene variants that have been found to be associated with giant cell arteritis that play a role in a person’s autoimmunity response. In addition, some infections (bacterial and viral) have been associated with GCA, but no specific infection has been identified.
Giant cell arteritis prevalence, or the total number of cases at a given time, is estimated to be 228 per 100,000 in USA. Using this data it is estimated that about 228,000 have GCA. With the aging of the US population, these estimates are likely to increase in coming years as it most commonly affects people over 50 years old (mostly over 65 years). It is more common in Caucasians, people of Nordic or northern European descent, and others in northern latitudes. Females are 2 to 3 times more likely to develop GCA than males in persons of northern European descent while there is no higher risk for females from Spain, Israel, Turkey, other Mediterranean countries and India. Specifically, about 20 per 100,000 people among whites in northern European populations are affected, 10 per 100,000 people affected among southern European populations, and about 1 per 100,000 people affected among American populations of Asian or African descent.
A diagnosis of giant cell arteritis (GCA) should be considered in anyone over 50 who experiences new headaches or a change in the pattern of existing headaches, sudden vision changes (particularly temporary or permanent vision loss in one eye), or pain in the jaw while chewing, known as jaw claudication. An unexplained fever, fatigue and other general symptoms may also be present. Abnormal findings during a physical exam such as tenderness, decreased pulse, or swelling of the temporal artery, may raise suspicion. These symptoms are especially concerning in people who have or have had polymyalgia rheumatica.
The initial evaluation usually includes:
If a diagnosis is still uncertain after these tests, further imaging may be needed to look for inflammation in larger arteries including:
To help guide diagnosis, the American College of Rheumatology (ACR) has proposed criteria that include age over 50, a new headache, abnormalities of the temporal artery on exam, an ESR of at least 50 mm/hour and findings from a temporal artery biopsy. Having at least three of these increases the likelihood of GCA.
International guidelines from the European League Against Rheumatism (EULAR) recommend ultrasound as the first diagnostic test, with biopsy as an option when ultrasound is unavailable or inconclusive.
Once treatment begins, usually with high-dose steroids, it is important to monitor both the effectiveness of the treatment and any side effects. Blood tests for ESR and CRP are repeated regularly to assess disease activity and detect relapses. People are monitored for ongoing or new symptoms, such as headaches, jaw or tongue pain, visual disturbances, muscle stiffness and side effects of steroid medications.
Follow-up care is essential and usually scheduled at regular intervals: one week, three weeks, six weeks, three months, nine months and twelve months after diagnosis. The purpose of these visits is to track disease progress, evaluate complications and adjust treatment as needed. While some doctors recommend bone and chest X-rays every two years, there is no evidence that this is useful.
In some people, if both the biopsy and imaging tests are negative but symptoms strongly suggest GCA and other conditions have been ruled out, doctors may still diagnose and treat GCA based on clinical judgment.
Treatment
The treatment of giant cell arteritis (GCA) aims to quickly control inflammation, relieve symptoms and prevent serious complications such as vision loss. Treatment is personalized, balancing the need to control symptoms with the goal of minimizing harm from medication (weighing risks and benefits).
The standard treatment approach includes the use of corticosteroids (steroids) but newer medications have been introduced to reduce long-term use of corticosteroids.
As soon as GCA is diagnosed, treatment with corticosteroids should begin right away. Delaying therapy, even to wait for test results like biopsies or scans, is not recommended due to the risk of permanent damage, especially to vision or the brain.
Most people start with prednisolone, an oral corticosteroid. In severe cases, if there are symptoms affecting vision or the brain, methylprednisolone may be given intravenously (through a vein) initially, followed by oral treatment. Corticosteroids usually begin at higher doses and are gradually reduced (a process called tapering) over time to minimize side effects and prevent the disease from returning (relapse).
Most people improve with the treatment within 2 to 4 weeks. However, treatment often continues for up to two years, and in some cases longer.
Some symptoms, especially headaches, may return during this tapering period and many may also develop symptoms of polymyalgia rheumatica. These symptoms may be treated with slight increases in the doses of corticosteroids or with an immune-suppressing drug called methotrexate (Trexall).
Long-term use of corticosteroids can lead to serious side effects. More than 80% of people report issues, particularly bone loss. Doctors may ask for exams to monitor bone density and might prescribe calcium and vitamin D supplements or other medications to help prevent bone loss. To reduce this risk, doctors may recommend medications like bisphosphonates, which strengthen bones and reduce fracture risk.
People taking methotrexate may also be given folate or leucovorin to protect healthy cells and reduce side effects.
Because of the long-term corticosteroid risks, researchers have developed newer therapies that reduce the need for corticosteroids, known as corticosteroids-sparing treatments:
Some people may also need medications like sulfamethoxazole-trimethoprim or dapsone to prevent infections if their immune system is suppressed.
Relapses can happen, especially as corticosteroids doses are reduced. When this occurs, treatment may involve:
If the blood vessels in the brain (cerebral arteries) or neck (carotid arteries) are severely narrowed or blocked, the doctor may prescribe aspirin to help prevent blood clots. This is usually combined with medications like corticoids or corticoid-sparing treatments.
People with other serious blood vessel problems like an aortic aneurysm (a bulge in a major artery) or narrowing of arteries that limit blood flow to the limbs or organs may need surgery with consultation with a vascular surgeon and a rheumatologist.
Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government web site.
For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:
Toll-free: (800) 411-1222
TTY: (866) 411-1010
Email: [email protected]
Some current clinical trials also are posted on the following page on the NORD website: https://rarediseases.org/for-patients-and-families/information-resources/news-patient-recruitment
For information about clinical trials sponsored by private sources, contact: www.centerwatch.com
For more information about clinical trials conducted in Europe, contact: https://www.clinicaltrialsregister.eu/
TEXTBOOKS
Langford CA, Fauci AS. The Vasculitis Syndromes. In: Jameson J, Fauci AS, Kasper DL, Hauser SL, Longo DL, Loscalzo J. eds. Harrison’s Principles of Internal Medicine, 20e New York, NY: McGraw-Hill; 2018.
JOURNAL ARTICLES
Ehlers L, Askling J, Bijlsma H, et al. 2018 EULAR recommendations for a core data set to support observational research and clinical care in giant cell arteritis. Ann Rheum Dis. 2019 Mar 21. pii: annrheumdis-2018-214755.
Wojczal J, et al. Advantages in diagnosis of giant cell arteritis by ultrasound. Postepy Dermatol Alergol. 2019. Feb;36(1):25-28.
Coath F, Gillbert K, Griffiths B, et al. Giant cell arteritis: new concepts, treatments and the unmet need that remains. Rheumatology (Oxford). 2018 Nov 12; key326
Ninan J, Lester S, Hill C. Giant cell arteritis, beyond temporal artery biopsy and steroids. Internal medicine journal. 2017. Nov;47(11):1228-1240.
Koster M, Warrington K. Giant cell arteritis pathogenic mechanisms and new potential therapeutic targets. BMC Rheumatol. 2017. Nov 28;1:2.
Francis C. Giant cell arteritis. J Neuroophthalmol. 2016.Mar;36(1): e2-4.
Nesher G. The diagnosis and classification of giant cell arteritis. J Autoimmun. 2014. Feb-Mar;48-49:73-5
Smith J, Swanson J. Giant cell arteritis. Headache. 2014. Sep;54(8):1273-89.
Li KJ, Semenov D, Turk M, Pope J. A meta-analysis of the epidemiology of giant cell arteritis across time and space. Arthritis Res Ther. 2021;23(1):82. Published 2021 Mar 11. doi:10.1186/s13075-021-02450-w
Sharma A, Mohammad AJ, Turesson C. Incidence and prevalence of giant cell arteritis and polymyalgia rheumatica: A systematic literature review. Semin Arthritis Rheum. 2020;50(5):1040-1048. doi:10.1016/j.semarthrit.2020.07.005
INTERNET
Salvarani C & Muratore F. Treatment of giant cell arteritis. UpToDate. Last updated May 30, 2025. https://www.uptodate.com/contents/treatment-of-giant-cell-arteritis Accessed June 26, 2025.
Salvarani C & Muratore F. Diagnosis of giant cell arteritis. UpToDate. Last updated Nov 6, 2024. https://www.uptodate.com/contents/diagnosis-of-giant-cell-arteritis#H24 Accessed June 26, 2025.
National Institute of Arthritis and Skin and Musculoskeletal Diseases. Polymyalgia Rheumatica and Giant Cell Arteritis. February 2022. https://www.niams.nih.gov/health-topics/polymyalgia-rheumatica-giant-cell-arteritis Accessed June 26, 2025.
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