HistoryThis section has been translated automatically.
Hutchinson 1890; Horton 1934
DefinitionThis section has been translated automatically.
Chronic, segmental, granulomatous, obliterating "large vessel" vasculitis, which usually manifests itself on the carotid artery and its outlets. Preferential infestation of the temporalis, ophthalmic, facial, occipital, lingual and maxillary arteries with corresponding symptoms. There are no large vessels on the skin; however, the disease can affect vessels supplying the skin areas.
Remark: 50% of the patients also suffer from polymyalgia rheumatica!
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Occurrence/EpidemiologyThis section has been translated automatically.
Incidence: 9/1,00,000 population, in < 50-year-olds: <5/100,000; Dec 6: 40/100,000; Dec 7: 40/100,000 population.
The annual incidence in northern Europe is >17/100,000 in persons > 50 years of age. In southern Europe, the incidence is <12/100,000.
EtiopathogenesisThis section has been translated automatically.
Unknown! A T-cell-dependent (auto)immune event with genetic predisposition, possibly triggered by infection, is discussed.
Age > 50 years
Pathogenetically, it is a granulomatous giant cell arteritis in the media and adventitia of the affected arterial segments with consecutive sclerotic vessel wall senescence.
ManifestationThis section has been translated automatically.
Age group > 50 years; preferred by women (75%), mostly Caucasians.
Clinical featuresThis section has been translated automatically.
Sudden onset of disease!
General symptoms: initially: throbbing, temporal headache, fever, feeling sick, arthralgias, myalgias, morning stiffness and weight loss. 50% of patients have concomitant polymyalgia rheumatica. Furthermore: orthostatic dizziness (40%); seizures and hemiparesis (<10%); nonspecific skin involvement: erythema nodosum or urticaria (15%).
Arteriitis cranialis with variable often simultaneous pattern of involvement (Note: A. temporalis affected in most cases):
- Temporal artery: often severe unilateral or bilateral throbbing headache, especially temporal and frontal (50%), not infrequently pain when chewing (jaw claudication; masseteric claudication), redness in the area of the cord-like thickened temporal artery: not infrequently small or large necroses of the skin (and galea) in the catchment area of the temporal artery may occur.
- Ophthalmic artery (30% of cases): Visual disturbances, amaurosis fugax, risk of blindness; possibly double vision (diplopia).
- Lingual artery: painful redness, blisters, necrosis on the tongue.
- Brachial artery: claudication of the arms (50%); blood pressure differences of more than 30 mm Hg in both arms (64%); absent pulses (50%).
- A. femoralis: claudication of the lower extremities (10%);
- Aorta: aortic aneurysm or insufficiency (10%) - aortic arch syndrome -.
ImagingThis section has been translated automatically.
- Evaluation of the temporal artery in comparison to the sides (hardened, tortuous arteries, palpable, laterally differentiated pulsations).
- Doppler sonographic examination of the head arteries (including exclusion of high-grade internal carotid stenoses).
- Colour duplex of the temporal arteries (wall thickening, pulsations).
LaboratoryThis section has been translated automatically.
- Acute phase reaction:
- Severe increase in BSG (often > 80 mm/hour; normal in 5% of patients)
- C-reactive protein (increase in > 90%, as a progression parameter more meaningful than BSG
- eosinophilia, leucocytosis
- Increase of the alpha-2-globulin fraction (norm: ~ 7.1 - 11.8% from the age of 14 years; reference value of the central laboratory of the University Hospital of Heidelberg), e.g. of the haptoglobin (norm: 0.3 - 2.0 g/dL), as well as if necessary of the VLDL, alpha-2-macroglobulin and/or the coeruloplasmin
- Increase of the fibrinogen i.P. (Norm: 200-450 mg/dL)
- Infectious anaemia (haemoglobin < 13 mg/dL in men, < 12 mg/dL in non-pregnant women);
- There is no positive rheumatoid factor or ANA and ANCA evidence.
HistologyThis section has been translated automatically.
|Concerning arteries of the subcutis and deeper tissue|
|Perivascular, intramural and/or intraluminal leukocytoclasia|
|Damage to endothelial cells|
|Fibrin in/around vessel walls|
|Perivascular extravasation of erythrocytes|
|No edema in the papillary dermis|
|Characteristic giant cells, most often near the internal elastic membrane|
|Catholic. Changes limited to vascular, not extravascular, interstitial or soft tissue granulomas|
|Plasma cells or fibrosclerosis to a variable degree|
|Reorganisation due to lymphocytic vasculitis|
DiagnosisThis section has been translated automatically.
Clinical diagnosis according to criteria of the American College of Rheumatology (ACR) for the diagnosis of the temporalis (Hunder et al. 1990):
- Age: > 50 years
- secondary headache
- Abnormal temporal arteries (pressure bulge, weakened pulsation).
- BSG > 50 mm in the first hour.
- Histological changes in biopsy of the temporal artery (important: segmental vasculitis "skip lesions"; possibly several biopsies necessary! Before that, arterial Doppler to exclude flow sounds!)
If 3 of 5 criteria are met, a sensitivity of 75-95%, a specificity of 90-93%, a positive predictive value of only 29% and a negative predictive value of 99% are achieved.
Furthermore, rheumatoid factor or antibodies against CCP (cyclic citrullinated peptide), CRP are to be determined.
If necessary, biopsy of the temporal artery (possibly on both sides, approx. 3 cm long segment due to segmental infestation). Note: Before biopsy, Doppler sonographic clarification of the arterial flow conditions.
Differential diagnosisThis section has been translated automatically.
Complication(s)This section has been translated automatically.
Internal therapyThis section has been translated automatically.
The therapeutic goal is to reduce the inflammation of the vascular wall. Indicators are the humoral symptoms of inflammation. Of crucial importance is the ocular symptomatology. Flow in the central retinal artery should be measured and can be included as a therapeutic control symptom.
- Glucocorticoids: Prednisone equivalents in an initial dosage of 1.0-1.5 mg/kg bw for 7-14 days, then reduction by 10 mg/day up to a dosage of 25-40 mg/day for 4 weeks; further reduction by 5 mg/week up to a maintenance dose of < 10 mg/day p.o. for 1 year. Subsequent therapy depending on the clinic (acute phase reaction as indicator of inflammatory symptoms).
- Already in case of unilateral ocular symptoms (visual disturbances up to blindness), higher prednisone equivalents (1.5-2.0 mg/kg bw/day) should be started. If this therapy regime is not sufficient (recurrences during treatment), an additive therapy with cyclophosphamide (2 mg/kg bw/day) according to the standard scheme of Fauci is necessary. This can still benefit about 4% (!) of glucocorticoid-resistant patients. Methotrexate can be administered as an alternative to cylophosphamide.
- Non-steroidal anti-inflammatory drugs: Complementary to the therapy with glucocorticoids NSAIDs in medium dosage.
- In 2017, the IL-6 receptor inhibitor tocilizumab (e.g. Actemra®) was approved as a biologic that demonstrated efficacy in RZA and showed superiority to glucocorticosteroids. The drug has already been approved for rheumatoid arthritis (RA) since 2009. Approved.
Progression/forecastThis section has been translated automatically.
Note(s)This section has been translated automatically.
The arteriitis temporalis is synonymous with the term giant cell arteriitis and is compared to the Takayasu arteriitis. This is incorrect, since the histological substrate of both entities is the "giant cell arteritis" and thus no difference is expressed.
LiteratureThis section has been translated automatically.
- Buttgereit F et al. (2016) Polymyalgia Rheumatica and Giant Cell Arteritis: A Systematic Review.
- Campbell FA et al (2003) Scalp necrosis in temporal arteritis. Clin Exp Dermatol 28: 488-490.
- Healy LA, Wilske KR (1978) The systemic manifestations of temporal arteritis. Grune and Stratton, New York.
- Hamidou MA et al (2003) Temporal arteritis associated with systemic necrotizing vasculitis. J Rheumatol 30: 2165-2169.
- Hernandez-Garcia C et al (1994) Methotrexate treatment in the management of giant cell arteritis. Scand J Rheumatol 23:295-298
- Horton BT, Magath TB, Brown GE (1934) Arteritis of temporal vessels. Arch Intern Med 53: 400
- Hunder GG et al (1990) The American College of Rheumatology 1990 criteria for the classification ofgiant
cell arteritis. Arthritis Rheum 33:1122-1128
- Hutchinson J (1890) Diseases of arteries. Arch Surg 1: 323
Mehta P et al (2021) Giant Cell Arteritis and COVID-19: Similarities and Discriminators. A Systematic Literature Review. J Rheumatol 48: 1053-1059.
Mejren A et al (2022) Large-vessel giant cell arteritis after COVID-19 vaccine. Scand J Rheumatol 51:154-155.
Mursi AM et al.(2022) A Case Report of Post COVID19 Giant Cell Arteritis and Polymyalgia Rheumatica With Visual Loss. Clin Med Insights Case Rep 15:11795476221088472.
- Pfadenhauer K, Weber H (2003) On the current status of ultrasound diagnosis of temporal arteritis. Neurologist 74: 683-690
- Ratzinger G et al (2015) The vasculitis wheel-an algorithmic approach to cutaneous vasculitides. JDDG 1092-1118
- Salvarani C et al (2002) Polymyalgia rheumatica and giant-cell arteritis. N Engl J Med 347: 261-271
- Takayasu M (1908) A case of strange anastomosis of the central vessels of the retina. J Jap Ophthalm Soc 12: 554
- Trautvetter U et al (1992) Larval progressive form of giant cell arteritis-A dermatologic challenge. Z Hautkr 67: 822-826
- Weyand CM et al (2003) Medium- and large-vessel vasculitis. N Engl J Med 349: 160-169
Incoming links (25)Aortic arch arteritis; Aortic arch syndrome; Arteritis, eosinophilic; Arteritis, giant cell arteritis; Autoimmune diseases; Brachiocephalic arteritis; Cranial arteritis; Cranial giant cell arteritis; Eosinophilia and skin; Eosinophilic granulomatosis with polyangiitis; ... Show all
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