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IHSDiagnosisICD-10
6.4.1Headache attributed to giant cell arteritis (GCA) [M31.6]G44.812  
Previously used terms Temporal arteritis, Horton's disease

Diagnostic criteria:

  1. Any new persisting headache fulfilling criteria C and D
  2. At least one of the following:
    1. swollen tender scalp artery with elevated erythrocyte sedimentation rate (ESR) and/or C reactive protein (CRP)
    2. temporal artery biopsy demonstrating giant cell arteritis
  3. Headache develops in close temporal relation to other symptoms and signs of giant cell arteritis
  4. Headache resolves or greatly improves within 3 days of high-dose steroid treatment

Comments:

Of all arteritides and collagen vascular diseases, giant cell arteritis is the disease most conspicuously associated with headache (which is due to inflammation of head arteries, mostly branches of the external carotid artery). The following points should be stressed:
  • the variability in the characteristics of headache and other associated symptoms of GCA (polymyalgia rheumatica, jaw claudication) are such that any recent persisting headache in a patient over 60 years of age should suggest GCA and lead to appropriate investigations;
  • recent repeated attacks of amaurosis fugax associated with headache are very suggestive of GCA and should prompt urgent investigations;
  • the major risk is of blindness due to anterior ischaemic optic neuropathy, which can be prevented by immediate steroid treatment;
  • the time interval between visual loss in one eye and in the other is usually less than 1 week;
  • there are also risks of cerebral ischaemic events and of dementia;
  • on histological examination, the temporal artery may appear uninvolved in some areas (skip lesions) pointing to the necessity of serial sectioning;
  • duplex scanning of the temporal arteries may visualise the thickened arterial wall (as a halo on axial sections) and may help to select the site for biopsy.

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