Background
Main text
Changing epidemiology
GCA and PMR, two overlapping conditions
Cranial vs extra-cranial disease
Current diagnostic tools and diagnostic challenges
Typical cranial disease | |
New onset headache (mainly temporal) | |
Scalp pain | |
Jaw or tongue claudication | |
Acute visual deficits | |
Temporal artery abnormalities on physical examination | |
Anterior ischemic optic neuropathy or central retinal artery occlusion on ophthalmologic examination | |
Associated constitutional symptoms | |
Associated polymyalgia rheumatica symptoms | |
Associated anemia an elevated C reactive protein/erythrocyte sedimentation rate | |
Extra-cranial disease | |
Ischemic signs and symptoms of extremities, especially in the absence of other cardiovascular risk factors or emboligenic cardiopathy: | |
Limb claudication | |
Pulse asymmetry | |
Arterial pressure asymmetry | |
Peripheral arterial bruits | |
Distal necrosis or gangrene | |
Non-specific manifestations without evidence of infectious o neoplastic disease: | |
Fever | |
Weight loss | |
Fatigue/malaise | |
Unexplained anemia | |
Polymyalgia rheumatica that relapses or responds poorly to standard glucocorticoid therapy | |
Polymyalgia rheumatica with associated ischemic manifestations | |
Detection of aneurysm or dissection of aorta and main branches along with raised inflammatory markers |
- Former term “temporal arteritis” might be misleading or confounding, as virtually any large or medium-sized artery may be affected. | |
- GCA may present with isolated extra-cranial involvement. | |
- PMR may be present before, during or after diagnosis of GCA has been established, and vice versa | |
- Comprehensive clinical assessment should include palpation of the temporal arteries as well as palpation and auscultation of extracranial vascular territories, including axillary and subclavian arteries, in order to look for any one-sided vascular stenosis. Arterial pressures should also be measured in all four limbs. | |
- Temporal artery biopsy is the gold standard for the diagnosis of GCA with cranial manifestations. However, the temporal artery yield decreases significantly in patients presenting with extra-cranial GCA. | |
- Temporal artery biopsy is positive in patients with cranial manifestations even several weeks after the onset of glucocorticoids. | |
- When there is a very suggestive clinical picture and a positive imaging test, presumptive diagnosis of GCA may be established, thus obviating temporal artery biopsy. | |
- Glucocorticoid therapy should be started immediately in patients with a high clinical suspicion of GCA, even before histologic confirmation or imaging tests are available. | |
- When ophthalmologic symptoms are present, high doses of glucocorticoids are recommended. | |
- After diagnosis of GCA has been established, it may be advisable to use CT-angiography or FDG-PET/CT for screening of large-vessel involvement. | |
- Large-vessel complications (aneurysm and dissection) may develop several years after initial diagnosis of GCA, therefore long-term close follow-up is advised. |