Emergency: requires immediate attention
Giant cell arteritis
Alerts and Notices
Synopsis
Patients may present with systemic symptoms such as a new temporal headache, fever, anorexia, weight loss, morning stiffness in the shoulder or neck, myalgia, fatigue, and anemia. There may be elevated ESR or C-reactive protein, temporal artery tenderness to palpation, or decreased arterial pulsation unrelated to arteriosclerosis. Patients may have scalp tenderness, visual disturbances (amaurosis fugax), jaw or tongue claudication or difficulty chewing, or upper cranial palsies.
GCA and polymyalgia rheumatica (PMR) have a significant clinical association: 16%-21% of cases of PMR are associated with GCA, and 40%-60% of patients diagnosed with GCA also have PMR.
The most serious complication of GCA is sudden blindness, a result of anterior ischemic optic neuropathy caused by ischemia secondary to inflammation of the ophthalmic artery supplying the optic nerve. Some patients may experience transient vision loss. All patients with suspected GCA should have temporal artery biopsy. Pathologic findings include a predominance of mononuclear cell infiltration or granulomatous inflammation, usually with multinucleated giant cells.
Scalp necrosis is a rare but potentially life-threatening complication.
The 2022 American College of Rheumatology (ACR) / European Alliance of Associations for Rheumatology (EULAR) classification criteria for GCA when a diagnosis of medium-vessel or large-vessel vasculitis has been made:
A patient aged 50 years or older; 6 or more of the following points are needed for diagnosis:
Clinical criteria
- Morning stiffness in the shoulders or neck, jaw or tongue claudication, new temporal headache, scalp tenderness, abnormal clinical examination of the temporal artery (such as diminished pulse, tenderness, or a hard, cord-like texture) (+2 each)
- Sudden visual loss (+3)
- ESR ≥ 50 mm/hour or C-reactive protein ≥ 10 mg/L (+3)
- Positive temporal artery biopsy or temporal artery halo sign on ultrasound (+5)
- Bilateral axillary luminal damage (as seen on angiography, ultrasound, or fluorodeoxyglucose-positron emission tomography [FDG-PET]; +2)
- FDG-PET uptake throughout the aorta (+2)
Codes
ICD10CM:M31.6 – Other giant cell arteritis
SNOMEDCT:
400130008 – Temporal arteritis
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Diagnostic Pearls
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Differential Diagnosis & Pitfalls
- Aortic dissection
- Takayasu arteritis
- Kawasaki disease
- Carotid stenosis
- Cerebrovascular disease
- Arteriovenous fistula
- Intracranial neoplasm
- Sepsis
- Acute angle-closure glaucoma
- Granulomatosis with polyangiitis
- Iritis / uveitis
- Migraine headaches
- PMR
- Retinal artery occlusion (branch, central)
- Retinal vein occlusion (branch, central)
- Rheumatoid arthritis
- Polyarteritis nodosa
- Amyloidosis
- VEXAS syndrome
- Endocarditis
Best Tests
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Management Pearls
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Therapy
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Drug Reaction Data
Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.Subscription Required
References
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Last Reviewed:02/25/2019
Last Updated:02/08/2023
Last Updated:02/08/2023
Emergency: requires immediate attention
Patient Information for Giant cell arteritis
Overview
Giant cell arteritis, or temporal arteritis, is inflammation of the lining of the arteries. It typically affects the head and causes frequent headaches. It is most common among people over the age of 50. If left untreated, giant cell arteritis can lead to more serious conditions, including loss of vision and stroke.Who’s At Risk
Risk factors include:- Age over 50
- Women are twice as likely to develop giant cell arteritis
- Most common among whites in Northern Europe and of Scandinavian descent
- The condition polymyalgia rheumatica increases your risk of developing giant cell arteritis
- Family history of giant cell arteritis
Signs & Symptoms
The most common symptom of giant cell arteritis is a severe headache on both sides of the head on the temples. Other symptoms can include:- Scalp tenderness
- Neck pain and jaw pain especially when opening the mouth
- Fever
- Muscle ache and fatigue
- Loss of appetite and weight loss
- Impaired vision
- Sudden loss of vision in one eye
Self-Care Guidelines
Discuss side effects of medications used to treat giant cell arteritis with your health care provider. Schedule regular doctor visits.Your doctor may recommend daily aspirin to reduce your risk of stroke.
When to Seek Medical Care
Contact your health care provider if you develop a persistent headache or have any of the symptoms listed above, as treatment should be started as soon as possible.Treatments
A high dose of corticosteroids (prednisone) is the treatment for giant cell arteritis. Symptoms should improve within a few days of starting treatment. Your health care provider will slowly reduce the dosage depending on your symptoms. Treatment with corticosteroids may last one to two years.Emergency: requires immediate attention
Giant cell arteritis