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Emergency: requires immediate attention
Giant cell arteritis
Other Resources UpToDate PubMed
Emergency: requires immediate attention

Giant cell arteritis

Contributors: Michael W. Winter MD, Ryan Hoefen MD, PhD, Susan Burgin MD, Paritosh Prasad MD
Other Resources UpToDate PubMed

Synopsis

Giant cell arteritis (GCA) is a systemic vasculitis affecting medium- to large-sized arteries, including the aorta and the extracranial branches of the carotid artery. GCA is rarely found in individuals younger than 55, and the mean age for disease presentation is 76.

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)
Laboratory, imaging, and biopsy criteria
  • 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

Look For

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

  • Aortic dissection
  • Takayasu arteritis
  • Kawasaki disease
  • Carotid artery stenosis
  • Cerebrovascular disease
  • Arteriovenous fistula
  • Intracranial neoplasm
  • Bacterial sepsis
  • Acute angle-closure glaucoma
  • Granulomatosis with polyangiitis
  • Iritis / Uveitis
  • Migraine headache
  • Polymyalgia rheumatica
  • Retinal artery occlusion (Branch retinal artery occlusion, Central retinal artery occlusion)
  • Retinal vein occlusion (Branch retinal vein occlusion, Central retinal vein occlusion)
  • Rheumatoid arthritis
  • Polyarteritis nodosa
  • AL amyloidosis
  • VEXAS syndrome
  • Subacute bacterial 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.

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References

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Last Reviewed:01/23/2023
Last Updated:02/08/2023
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Emergency: requires immediate attention
Patient Information for Giant cell arteritis
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Emergency: requires immediate attention
Giant cell arteritis
A medical illustration showing key findings of Giant cell arteritis
Copyright © 2024 VisualDx®. All rights reserved.