Temporal arteritis

Created 13/05/2023, last revision 21/05/2023

temporal arteritis (TA), also called giant cell arteritis (GCA) is a systemic inflammatory vasculitis of medium and large-sized arteries occurring in elderly adults
- the temporal artery is most commonly involved
- aorta and the subclavian, iliac, ophthalmic, occipital, and vertebral arteries can be affected too
it is more common in women and mostly affects individuals > 50 years of age
the most prominent symptoms are headaches, jaw claudications, and visual disturbances (ischemic optic neuropathy – ION)
if untreated, it can result in severe complications (most usually blindness)
TA is sometimes associated with polymyalgia rheumatica (both entities are considered a continuum of a pathological process and temporal arteritis is a sign of advanced inflammation)

Etiopathogenesis

large and/or medium-sized arteries inflammation
- external carotid artery (ECA) and its branches (superficial temporal artery – STA) are most commonly involved
- ICA and intracranial segments or other major arteries are rarely affected
the exact cause is unknown; several risk factors were identified
- age – rarely affects those under 50; most patients are between 70 and 80
- sex – women have double risk
- race and geographic region – more common among white people in Northern European populations
- polymyalgia rheumatica – having polymyalgia rheumatica increases the risk of developing temporal arteritis
- family history
histology
- a panarteritis with the formation of giant cell granulomas, often near the disrupted lamina elastica internal
- necrosis of the vascular smooth muscle, proliferation of the intima, and thrombosis
- the vessel wall is infiltrated by activated macrophages and CD4T lymphocytes (producing Th-1 cytokines)
vessel involvement is segmental, with narrowing or even obliteration of the vessel lumen

general symptoms are dominated by anorexia, weight loss, fever (~15%), night sweats, depression, fatigue
frequent headache (40-90% of patients; may be severe)
elevated inflammatory markers (CRP, ESR)
myalgia, stiffness in the neck, shoulders, or hips (related to polymyalgia rheumatica, which may be present in up to 50% of cases)

scalp tenderness, pain and tenderness over the temples; the skin may be red
the temporal artery is swollen and sensitive to palpation, with absent pulsations, palpable nodes may be present
sometimes arteritis is occult, and the first symptoms are headache and unilateral blindness

local findings that are evident on clinical examination (inspection, palpation) are described above

if there is a reasonable clinical suspicion, it is not advisable to delay treatment
the rapid regression of subjective complaints after the start of corticosteroid therapy becomes an important diagnostic criterion (in other autoimmune diseases, the relief is not so rapid and significant)

Diagnostic criteria In the presence of 3 out of 5 criteria, the sensitivity is ~ 93%
age > 50 years (average age 69 years) localized headache STA local pathology (swelling, enlarged and tortuous vessel with palpation tenderness, reduced pulsations) ↑CRP, ESR (> 50 mm/h) positive biopsy (necrosis, giant cell infiltrate)

in the presence of myalgia, consider polymyalgia rheumatica
other vasculitides
- Takayasu’s arteritis
- polyarteritis nodosa
- microscopic angiitis

because of the risk of vision loss, start treatment ASAP (before temporal arteritis is confirmed by biopsy)
corticosteroids are the first-line choice, may be combined with imunosuppresive drugs (combination allows lower maintenance doses of corticosteroids)
- a high initial dose helps to control symptoms quickly
- a lower steroid dose given over a longer period of time should prevent relapse
- precautions are needed for long-term use of corticosteroids to avoid side effects (osteoporosis, peptic ulcer, hypokalemia, diabetes) → see here
the duration of treatment is guided by the clinical course and CRP and ESR levels
the ESR decline is usually rapid, sometimes with a latency of 3-5 days

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antiplatelet therapy (ASPIRIN 100mg daily) to reduce the risk of a stroke or heart attack

loss of vision in one or, rarely, both eyes; usually permanent
aortic aneurysm – this complication can occur even years after the diagnosis of giant cell arteritis; monitor aorta with annual chest X-rays or other imaging tests, such as ultrasound and CT
stroke – an uncommon complication of giant cell arteritis