Temporal arteritis

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Temporal arteritis
The arteries of the face and scalp.
ICD-10 M31.5
ICD-9 446.5
OMIM 187360
DiseasesDB 12938
MeSH D013700

Temporal Arteritis Microchapters

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor-In-Chief: Cafer Zorkun, M.D., Ph.D. [2]

Synonyms and keywords: GCA; giant cell arteritis; cranial arteritis; Horton's disease; Horton disease; Horton's arteritis; Horton syndrome; Horton's syndrome; granulomatous arteritis; polymyalgia arteritica

Overview

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.

This diagnosis should be considered in any patient over the age of 50 with the new onset of headache, particularly is the erythrocyte sedimentation rate is elevated.

Prompt treatment with steroids is a medical emergency to reduce the risk of blindness.

Pathophysiology

The damage to the vasuclature is mediated by an attack on the internal elastica lamina by activated CD4+ T helper cells. This occurs in repsonse to the presentation of an antigen by macrophages. The inciting antigen has not been identified.

Because the disease involves only arteries with internal elastic lamina, the aortic arch and its branches are often involved. Intracranial arteries do not have internal elastic lamina and are not involved. The distribution of involved arteries are as follows:

Commonly involved sites:

Cervicocephalic arteries: carotid artery and vertebral artery. The vertebral artery is involved as frequently as the temporal artery in fatal cases. Involvement of the basilar artery is rare.

Intraorbital branches: Posterior ciliary artery and ophthalmic artery.

External common, external, and internal carotid artery involvement: It is less common for proximal intracranial arteries to be involved.

External vertebral arteritis: It is less common though for the disease to extend more than 5 mm beyond the dural penetration.

Subclavian, axially and proximal brachial artery: There can be typical vasculitic lesions with long, smooth, lesions with tapered occlusions.

Coronary arteries: for a full discussion of the involvement of the heart in this disorder see the chapter on The Heart in Temporal Arteritis / Giant Cell Arteritis

Less commonly involved sites:

Descending aorta: Mesenteric, iliac, femoral and renal arteries are less often involved. In these cases there can be mesenteric ischemia, renal infarction, and ischemic mononeuropathy can occur.

Pulmonary artery

Diganosis

Diagnosis

Laboratory tests

Sedimentation rate is very high in most of the patients, but may be normal in approximately 20% of cases.

Radiology

Radiological examination of the temporal artery with ultrasound yields a halo sign. Contrast enhanced brain MRI and CT is generally negative in this disorder.

Treatment

Corticosteroids must be started as soon as the diagnosis is suspected (even before the diagnosis is confirmed by biopsy). Steroids do not prevent the diagnosis later being confirmed by biopsy, although certain changes in the histology may be observed towards the end of the first week of treatment and are more difficult to identify after a couple of months.[1]

A 3 day course of pulse steroids with 250 mg of IV solumedrol BID is critical in reducing the risk of permanent visual loss. If there is progression of visual loss on steroids, IV heparin can be administered to reduce the risk of thrombotic occlusion.

Treatment should not be deferred while waiting on the results of a temporal artery biopsy.

References

  1. Font RL, Prabhakaran VC (2007). "Histological parameters helpful in recognising steroid-treated temporal arteritis: an analysis of 35 cases". The British journal of ophthalmology. 91 (2): 204–9. doi:10.1136/bjo.2006.101725. PMID 16987903.

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