Temporal arteritis, also called giant cell arteritis (GCA) is an inflammatory disease of blood vessels (most commonly large and medium arteries of the head). It is therefore a form of vasculitis. The name comes from the most frequently involved vessel (temporal artery which branches from the external carotid artery of the neck). The alternative name (giant cell arteritis) reflects the type of inflammatory cell that is involved (as seen on biopsy).

The disorder may coexist (in one quarter of cases) with polymyalgia rheumatica (PMR), which is characterized by sudden onset of pain and stiffness in muscles (pelvis, shoulder) of the body and seen in the elderly. Other diseases related with temporal arteritis are systemic lupus erythematosus, rheumatoid arthritis and severe infections.

Symptoms

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It is more common in females and after 50 years of age. Below this age it is extremely rare.

Patients present with:

• fever
• headache
• tenderness and sensitivity on the scalp
• jaw claudication
• reduced visual acuity (blurred vision)
• acute visual loss (sudden blindness)

The inflammation may affect blood supply to the eye and blurred vision or sudden blindness may occur. In 76% of cases involving the eye the ophthalmic artery is involved causing anterior ischemic optic neuropathy. Loss of vision in both eyes may occur very abruptly and this disease is therefore a medical emergency.

Diagnosis

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Palpation of the head reveals sensitive and thick arteries with or without pulsation. Sedimentation rate is very high in most of the patients, but may be normal in approximately 20% of cases. The inflammation of the vessel must be demonstrated by removing a small part of the vessel (biopsy) and analysing it for giant cells infiltrating the tissue. Since the vessels are involved in a patchy pattern, there may be unaffected areas on the vessel and the biopsy might have been taken from these parts. So, a negative result does not definitely rule out the diagnosis.

Treatment

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Corticosteroids must be started as soon as the diagnosis is suspected (even before the diagnosis is confirmed by biopsy).

References

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• U of Iowa Prof. Sohan Singh Hayreh's Giant Cell Arteritis article
• Polymyalgia rheumatica article from National Institute of Arthritis and Musculoskeletal and Skin Diseases
• eMedicine Neurology article on Temporal/Giant Cell Arteritis

Rheumatology | Ophthalmology | Neurology | Medical emergencies

Arteriitis cranialis | Arteritis de células gigantes | Maladie de Horton | Dev hücreli arterit