Giant cell arteritis M31.6

Author: Prof. Dr. med. Peter Altmeyer

All authors of this article

Last updated on: 07.12.2022

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Aortic Arch Arteritis; Aortic Arch Syndrome; Arteriitis Giant cell arteriitis; Arteritis eosinophilic; Arteritis temporalis Horton; Bing-Horton disease; brachiocephalic arteritis; cranial arteritis; Cranial giant cell arteritis; giant-cell arteritis; Giant cell arteritis; Giant Cell Arteritis; Granulomatous vasculitis; Horton Magath Brown Syndrome; Horton's disease; Horton Syndrome; Martorell-Fabré Syndrome; pulseless disease; reversed isthmus stenosis syndrome; RZA; temporal arteritis; Temporal Arteritis

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Hutchinson 1890; Horton 1934

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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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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.

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Unknown! A T-cell-dependent (auto)immune event with genetic predisposition, possibly triggered by infection, is discussed.

Age > 50 years

Associations with viral infections (HBV, VZV, Parvovirus B19, SARS-CoV-2 - Mursi AM et al.2022) or Borrelia, Mycoplasma and Chlamydia (Chlamydia pneumoniae) are not certain.

Pathogenetically, it is a granulomatous giant cell arteritis in the media and adventitia of the affected arterial segments with consecutive sclerotic vessel wall senescence.

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Age group > 50 years; preferred by women (75%), mostly Caucasians.

Clinical features
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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 -.

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Apparative diagnostics:

  • 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).

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  • 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.

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Histopathological algorithm of giant cell arteritis (lowest common denominator: italic, leading symptoms: bold) varies according to Ratzinger et al. 2105
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
No eosinophiles
Plasma cells or fibrosclerosis to a variable degree
Reorganisation due to lymphocytic vasculitis

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Clinical diagnosis according to criteria of the American College of Rheumatology (ACR) for the diagnosis of the temporalis (Hunder et al. 1990):

  1. Age: > 50 years
  2. secondary headache
  3. Abnormal temporal arteries (pressure bulge, weakened pulsation).
  4. BSG > 50 mm in the first hour.
  5. 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 diagnosis
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Arteriosclerosis; thrombangitis obliterans; syphilis; in case of necrosis of the scalp, necrotizing zoster, erosive pustular dermatitis and bullous autoimmune diseases have to be considered; furthermore: ergotism (migraine therapeutics!);

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Circumscribed necrosis, blindness, apoplexy, myocardial infarction.

Internal therapy
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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.

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Mostly good response to glucocorticoids, usually complete remission after 6-24 months. Rare recurrences or chronic courses.

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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.

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  1. Buttgereit F et al. (2016) Polymyalgia Rheumatica and Giant Cell Arteritis: A Systematic Review.
    JAMA 315:2442-2458.
  2. Campbell FA et al (2003) Scalp necrosis in temporal arteritis. Clin Exp Dermatol 28: 488-490.
  3. Healy LA, Wilske KR (1978) The systemic manifestations of temporal arteritis. Grune and Stratton, New York.
  4. Hamidou MA et al (2003) Temporal arteritis associated with systemic necrotizing vasculitis. J Rheumatol 30: 2165-2169.
  5. Hernandez-Garcia C et al (1994) Methotrexate treatment in the management of giant cell arteritis. Scand J Rheumatol 23:295-298
  6. Horton BT, Magath TB, Brown GE (1934) Arteritis of temporal vessels. Arch Intern Med 53: 400
  7. Hunder GG et al (1990) The American College of Rheumatology 1990 criteria for the classification ofgiant
    cell arteritis. Arthritis Rheum 33:1122-1128
  8. Hutchinson J (1890) Diseases of arteries. Arch Surg 1: 323
  9. Mehta P et al (2021) Giant Cell Arteritis and COVID-19: Similarities and Discriminators. A Systematic Literature Review. J Rheumatol 48: 1053-1059.

  10. Mejren A et al (2022) Large-vessel giant cell arteritis after COVID-19 vaccine. Scand J Rheumatol 51:154-155.

  11. 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.

  12. Pfadenhauer K, Weber H (2003) On the current status of ultrasound diagnosis of temporal arteritis. Neurologist 74: 683-690
  13. Ratzinger G et al (2015) The vasculitis wheel-an algorithmic approach to cutaneous vasculitides. JDDG 1092-1118
  14. Salvarani C et al (2002) Polymyalgia rheumatica and giant-cell arteritis. N Engl J Med 347: 261-271
  15. Takayasu M (1908) A case of strange anastomosis of the central vessels of the retina. J Jap Ophthalm Soc 12: 554
  16. Trautvetter U et al (1992) Larval progressive form of giant cell arteritis-A dermatologic challenge. Z Hautkr 67: 822-826
  17. Weyand CM et al (2003) Medium- and large-vessel vasculitis. N Engl J Med 349: 160-169


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