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Giant Cell Arteritis: Causes, Symptoms & Treatment

Giant cell arteritis is an inflammatory disease of the blood vessels affecting mainly older adults. If untreated, it can lead to permanent vision loss.

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Things worth knowing about "Giant cell arteritis"

Giant cell arteritis is an inflammatory disease of the blood vessels affecting mainly older adults. If untreated, it can lead to permanent vision loss.

What is Giant Cell Arteritis?

Giant cell arteritis (GCA), also known as temporal arteritis or Horton disease, is a chronic inflammatory disease of medium and large blood vessels, particularly the arteries of the head and neck. It belongs to the group of systemic vasculitides – conditions involving inflammation of the vessel wall. The hallmark of the disease is the presence of so-called giant cells, large multinucleated immune cells found within the inflamed vessel wall. GCA almost exclusively affects people over the age of 50, with women being more commonly affected than men.

Causes

The exact causes of giant cell arteritis are not yet fully understood. It is believed to be an autoimmune reaction in which the immune system mistakenly attacks the walls of the body's own blood vessels. The following factors are discussed:

Genetic predisposition (e.g., certain HLA gene variants)
Infectious triggers (e.g., viral or bacterial infections as initiating factors)
Age-related changes in immune function
Association with polymyalgia rheumatica, an inflammatory muscle pain condition that frequently occurs alongside GCA

Symptoms

Giant cell arteritis can present with a wide variety of symptoms depending on which vessels are affected:

Severe, throbbing headache, especially at the temples
Tenderness or thickening of the temporal artery
Jaw pain when chewing (jaw claudication) due to reduced blood flow to the chewing muscles
Visual disturbances, including sudden and permanent loss of vision in one or both eyes
General symptoms such as fever, fatigue, weight loss, and night sweats
Pain and stiffness in the shoulders and hips (polymyalgia rheumatica)
In rare cases: stroke or heart attack due to involvement of larger arteries

Diagnosis

The diagnosis of giant cell arteritis is based on a combination of clinical examination, laboratory tests, and imaging:

Blood tests: Elevated inflammatory markers such as ESR (erythrocyte sedimentation rate) and CRP (C-reactive protein)
Ultrasound of the temporal artery: Detection of a characteristic inflammation sign known as the halo sign
Temporal artery biopsy: Tissue sample analyzed under the microscope for the presence of giant cells – considered the gold standard for diagnosis
PET-CT or MRI: Used to assess involvement of larger vessels (large vessel vasculitis)

Treatment

Treatment of giant cell arteritis must be initiated immediately upon diagnosis to minimize the risk of vision loss. The treatment of choice is corticosteroid therapy:

Prednisone at high doses (40–60 mg/day) is the standard treatment and typically leads to rapid symptom relief
In cases with threatened vision, treatment is started immediately with high-dose intravenous corticosteroids
The dose is gradually reduced over months to years to prevent relapse
Tocilizumab, a biologic drug that inhibits the inflammatory messenger IL-6, has been approved since 2017 as an add-on therapy and allows for faster tapering of corticosteroids
To prevent side effects of long-term corticosteroid use, calcium, vitamin D, and bisphosphonates are often prescribed to protect bone health

Prognosis and Disease Course

With early treatment, giant cell arteritis can be well controlled. The greatest risk is vision loss, which rarely occurs once corticosteroid therapy has been started. Many patients require maintenance therapy for several years. Relapses are possible, especially when medication is discontinued. Regular follow-up with a rheumatologist is strongly recommended.

References

Hellmich B. et al. - EULAR recommendations for the management of large vessel vasculitis. Annals of the Rheumatic Diseases, 2020; 79(1):19–30.
Jennette JC. et al. - 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis & Rheumatism, 2013; 65(1):1–11.
Stone JH. et al. - Trial of Tocilizumab in Giant-Cell Arteritis. New England Journal of Medicine, 2017; 377(4):317–328.

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