History

Large-vessel vasculitis is an umbrella term for diseases that affect the large arteries. The two main types are giant cell arteritis and Takayasu arteritis. There are other forms of large-vessel vasculitis that do not have specific names, such as idiopathic isolated aortitis.

Giant cell arteritis, also called temporal arteritis, and Takayasu arteritis affect the aorta and its major branches. Though both can cause blood vessel inflammation, they differ based on the age and presentation and vessels affected.

Epidemiology

Giant cell arteritis is the most common idiopathic systemic vasculitis, while Takayasu arteritis is more rare. In the United States, giant cell arteritis develops at a rate of 15 to 25 cases per 100,000 persons over age 50,¹ and Takayasu arteritis develops at a rate of 0.9 per million people.² Both giant cell arteritis and Takayasu arteritis affect women disproportionately to men. Giant cell arteritis develops in approximately 1% of women and 0.5% of men,³ and 80% to 90% of Takayasu arteritis cases develop in women.⁴

Ethnicity and advancing age are major risk factors for giant cell arteritis. The disease rarely, if ever, occurs before age 50, and more than 80% of patients with giant cell arteritis are older than 70.³ The highest incidence is in Scandinavian countries and among Americans of Scandinavian descent.³

With Takayasu arteritis, disease onset is typically before age 40.² Ethnicity is also a risk factor, as the greatest prevalence is in Asia where about 150 new cases of Takayasu arteritis occur each year in Japan.⁴

Large-Vessel Vasculitis Diagnosis & Presentation

Large-vessel vasculitis symptoms specific to giant cell arteritis come on subacutely. They may include³:

Fever
Fatigue
Weight loss
Headache
Jaw claudication
Transient visual loss
Permanent vision loss, especially in cases with prior transient visual loss
Proximal polyarthralgias and myalgias
Peripheral synovitis
Distal extremity swelling with pitting edema
Aneurysm of affected vessels
Stenosis
Aortic aneurysm or dilatation
Other large artery involvement

Less common manifestations of giant cell arteritis may include³:

Stroke
Upper respiratory tract symptoms
Maxillary and dental pain
Facial swelling
Throat pain
Tongue pain and macroglossia
Dysarthria
Sensorineural hearing loss
Breast mass
Female genital tract involvement
Mesenteric ischemia
Pericarditis

Takayasu arteritis also comes on subacutely. Symptoms of Takayasu arteritis may include⁴:

Limb claudication
Cyanosis
Lightheadedness
Arterial pain and tenderness
Weight loss
Low-grade fever
Fatigue
Arthralgias
Myalgias
Carotidynia
Absent or weak peripheral pulse(s)
Arterial bruit
Discrepant blood pressure between arms
Hypertension
Angina
Abdominal pain
Diarrhea
Gastrointestinal hemorrhage
Skin lesions
Dyspnea
Hemoptysis
Pulmonary hypertension
Vertigo
Syncope
Orthostasis
Headaches
Convulsions
Strokes

Visual impairment is a late manifestation of severe Takayasu arteritis.

Diagnostic Workup

A suspected diagnosis of large-vessel vasculitis may be confirmed by imaging or histology. A temporal artery biopsy is recommended to diagnose giant cell arteritis, while imaging is necessary for Takayasu arteritis.

Upon physical exam for suspected giant cell arteritis, look for:³

Abnormal pulses
Temporal artery abnormalities, such as thick, nodular, or tender frontal or parietal branches of the superficial temporal arteries
Bruits via auscultation
Heart murmur
Ocular findings via fundoscopy, such as cotton wool spots in the retina, a swollen pale disc and blurred margins in patients with vision loss from arteritic anterior ischemic optic neuropathy, or optic disc pallor in patients with permanent vision loss

Laboratory tests that may aid the diagnosis and assessment of giant cell arteritis include erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP). Note that neither the ESR nor the CRP is a specific biomarker for giant cell arteritis.

In addition, normochromic anemia is often present prior to therapy for giant cell arteritis.³

Elevated blood concentrations of hepatic enzymes, especially alkaline phosphatase, may also be noted.³

Physical exam for Takayasu arteritis should include:⁴

Measurement of blood pressure in all four extremities
Auscultation for bruits over the bilateral carotid, subclavian, axillary, renal, and femoral arteries and the abdominal aorta
Cardiac auscultation

Laboratory findings with Takayasu arteritis tend to be nonspecific, though ESR and

CRP may be elevated. Normochromic anemia may also be present.⁴

Imaging studies are essential for diagnosing Takayasu arteritis and determining the extent of vascular involvement. Positron emission tomography (PET) is often used in combination with computed tomography (PET-CT) or magnetic resonance (PET-MR).⁴

The American College of Rheumatology classification criteria can help distinguish one form of vasculitis from another. Patients have Takayasu arteritis if at least three of these six criteria are present. The criteria are⁵:

Age 40 or younger at disease onset
Limb claudication
Decreased pulsation of one or both brachial arteries
A difference of at least 10 mmHg in systolic blood pressure between the arms
Bruit over one or both subclavian arteries or the abdominal aorta
Arteriographic narrowing or occlusion of the entire aorta, its primary branches, or large arteries in the proximal upper or lower extremities not due to arteriosclerosis, fibromuscular dysplasia, or other causes

Differential Diagnosis

Differential diagnoses of giant cell arteritis may include:

Small- and medium-sized vessel vasculitis
Primary angiitis of the central nervous system
Nonarteritic anterior ischemic optic neuropathy
Infection

The differential diagnosis of Takayasu arteritis may include atherosclerotic, inflammatory, infectious, and/or genetic diseases that affect the large arteries. Additionally, other forms of large-vessel vasculitis/aortitis can present with clinical and radiographic features identical to Takayasu arteritis, including⁴:

Cogan syndrome
Relapsing polychondritis
Spondyloarthritis
Behçet syndrome
IgG4-related disease

While giant cell arteritis and Takayasu arteritis may be mistaken for one another, distinctions are usually made based upon the age of the patient and the distribution of lesions.

Large-Vessel Vasculitis Management
(Nonpharmacotherapy and Pharmacotherapy)

Glucocorticoid therapy (40-60 mg/day prednisone-equivalent) is the main treatment for remission induction in large-vessel vasculitis, including giant cell arteritis and Takayasu arteritis.⁶ Adjunctive therapy with IL-6 protein blockers, such tocilizumab or methotrexate, may be used in patients with refractory or relapsing disease and in those at increased risk for glucocorticoid-related adverse events or complications.⁶

Monitoring Side Effects, Adverse Events, Drug-Drug Interactions

Large-vessel vasculitis is chronic and tends to relapse and remit. As such, regular follow-up and monitoring of disease activity is recommended for all patients primarily based on symptoms, clinical findings, and ESR/CRP levels.⁶

Steroids confer their fair share of side effects, especially when used over the long-term. These side effects include increased infection risk, diabetes, hypertension, osteoporosis, easy bruising, and poor healing, and they need to be monitored and addressed if they occur.⁷

References

Lyons HS, Quick V, Sinclair AJ, et al. A new era for giant cell arteritis. Eye (Lond). 2020;34(6):1013-1026. doi:10.1038/s41433-019-0608-7

Alnabwani D, Patel P, Kata P, et al. The epidemiology and clinical manifestations of Takayasu arteritis: A descriptive study of case reports. Cureus. 2021;13(9):e17998. doi:10.7759/cureus.17998

Salvarani S, Muratore F. Clinical manifestations of giant cell arteritis. UptoDate. Accessed October 1, 2022. Last updated May 23, 2022.

Merkel PA. Clinical features and diagnosis of Takayasu arteritis. UptoDate. Accessed October 1, 2022. Last updated September 28, 2022.

Johnston SL, Lock RJ, Gompels MM. Takayasu arteritis: A review. J Clin Pathol. 2002;55(7):481-486. doi:10.1136/jcp.55.7.481

Hellmich B, Agueda A, Monti S, et al. 2018 Update of the EULAR recommendations for the management of large-vessel vasculitis. Ann Rheum Dis. 2020;79(1):19-30. doi:10.1136/annrheumdis-2019-215672

Cleveland Clinic. Vasculitis: 5 Drugs To Control This Mysterious Blood Vessel Illness. Updated April 3, 2017. Accessed October 1, 2022.

Author Bio

Denise Mann, MS, is a veteran freelance health writer in New York. Her work has appeared on HealthDay, among other outlets. She was awarded the 2004 and 2011 journalistic Achievement Awards from the American Society for Aesthetic Plastic Surgery. She was also named the 2011 National Newsmaker of the Year by the Community Anti-Drug Coalitions of America. She has also been awarded the Arthritis Foundation’s Northeast Region Prize for Online Journalism, the Excellence in Women’s Health Research Journalism Award, the Gold Award for Best Service Journalism from the Magazine Association of the Southeast, a Bronze Award from The American Society of Healthcare Publication Editors, and an honorable mention in the International Osteoporosis Foundation Journalism Awards. She was part of the writing team awarded a 2008 Sigma Delta Chi award for her part in a WebMD series on autism. Mann has a graduate degree from the Medill School of Journalism at Northwestern University in Evanston, Ill.