• Clinical science

Giant cell arteritis (Temporal arteritis…)

Abstract

Giant cell arteritis (GCA) is a type of vasculitis, which causes chronic inflammation of large and medium-sized arteries, secondary to autoimmune processes. It is most common in women over the age of 50. Classic localizations include the carotid artery and its major branches, as well as the aorta. Patients usually present with constitutional symptoms (e.g., fever, weight loss, night sweats, fatigue, and malaise), and new headaches. Depending on the involved arteries, throbbing pain over the temple region, jaw claudication, a tender and hardened temporal artery, temporary loss of vision (amaurosis fugax), and/or diplopia may occur. In addition to the clinical presentation and laboratory tests, which typically show a highly elevated erythrocyte sedimentation rate (ESR), temporal artery biopsy should always be performed to confirm the diagnosis. The classic histopathological findings are a mononuclear infiltration of the vessel wall with formation of giant cells. If GCA is suspected, immediate administration of glucocorticoids is essential to reduce symptoms and prevent severe complications, such as permanent vision loss and cerebral ischemia (e.g., transient ischemic attack and stroke). About half of patients with giant cell arteritis have coexisting polymyalgia rheumatica.

Epidemiology

  • Sex: >
  • Peak incidence: 70–79 years; rarely seen in patients < 50 years
  • More common among individuals of northern European descent

Women of advanced age are particularly prone to the disease!
References:[1][2]

Epidemiological data refers to the US, unless otherwise specified.

Etiology

  • Unknown; possible contributing factors are:
  • Association with polymyalgia rheumatica (PMR): 40–50% of patients with giant cell arteritis also have PMR

References:[1][#1200

Clinical features

  • Constitutional symptoms
    • Fever, weight loss, night sweats
    • Symptoms of anemia: fatigue and malaise
  • Symptoms of temporal artery inflammation (most commonly affected vessel)
    • New onset pulse-synchronous, throbbing, dull headache, typically located over the temples
    • Hardened and tender temporal artery
    • Tenderness of the temples or the scalp
    • Jaw claudication: jaw pain when chewing
  • Vision loss
    • Scintillating scotoma
    • Amaurosis fugax or permanent loss of vision
  • Diplopia
  • Symptoms of polymyalgia rheumatica (if both diseases are present)

About 50% of patients with giant cell arteritis also suffer from polymyalgia rheumatica!

References:[1][2]

Diagnostics

  • ACR criteria (3 of the 5 criteria are required)
    1. Age at disease onset ≥ 50 years
    2. Headaches
    3. Abnormalities of the temporal artery
    4. Elevated ESR (≥ 50 mm/h; see “Laboratory tests” below)
    5. Histopathological abnormalities in the temporal artery (see “Temporal artery biopsy” below)
  • Laboratory tests
  • Temporal artery biopsy (gold standard): mandatory in all patients
    • Extended biopsy sample (≥ 1 cm, ideally ≥ 2 cm)
    • Specificity: 100%
  • Duplex ultrasonography

References:[1][2][3][4][5]

Pathology

  • Panarteritis of the large and medium-sized arteries
  • Proliferation of the intima (and subsequent stenosis of the artery)
  • Fragmentation of the internal elastic lamina
  • Predominantly mononuclear infiltration of the vessel wall with formation of giant cells

References:[1]

Differential diagnoses

References:[6][1][2]

The differential diagnoses listed here are not exhaustive.

Treatment

Immediate administration of high-dose glucocorticoids is crucial to prevent permanent vision loss in patients with giant cell arteritis!
References:[7][1][8][9]

Complications

  • Permanent vision loss: ∼ 20–30% if giant cell arteritis is left untreated
  • Cerebral ischemia (e.g., transient ischemic attack and stroke): < 2% of cases
  • Thoracic artery arteritis (aortic arch syndrome) due to thoracic artery involvement → increased risk of thoracic artery aneurysm and dissection.
    • Annual chest x-ray screening recommended for up to 10 years after the diagnosis of GCA

References:[7][9]

We list the most important complications. The selection is not exhaustive.