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Revised Criteria for the Classification of Giant-Cell Arteritis (GCA)

Giant Cell Arteritis (GCA) is a systemic vasculitis of the medium and large sized vessels with a tendency to involve extracranial branches of the carotid arteries.

1990 ACR Criteria for the Classification of Giant-Cell Arteritis (GCA)

Three of the following five criteria were required to meet American College of Rheumatology (ACR) classification criteria for giant-cell arteritis:

  1. Age 50 years or older,
  2. New-onset localized headache,
  3. Temporal artery tenderness or decreased temporal artery pulse,
  4. Erythrocyte sedimentation rate of at least 50 mm/h, and
  5. Abnormal artery biopsy specimen characterized by mononuclear infiltration or granulomatous inflammation.

These criteria have a reported sensitivity of 93.5% and a reported specificity of 91.2% for the classification of giant-cell arteritis compared with other vasculitides.

 

Revised ACR Criteria for the Classification of Giant-Cell Arteritis (GCA)a

Score Entry
N/A Age at onset ≥50 years old
Absence of exclusion criteriab
Domain I
1 New onset localized headachec 1p
1 Sudden onset of visual disturbancesc 1p
2 Polymyalgia Rheumatica (PMR) 2p
1 Jaw Claudicationc 1p
2 Abnormal temporal arteryd up to 2p
Domain II
1 Unexplained fever and/or anaemia 1p
1 ESR ≥50 mm/houre 1p
2 Compatible pathologyf up to 2p

a In the presence of 3 points or more out of 11 with at least one point belonging to domain I along with all entry criteria, the diagnosis of Giant cell arteritis can be established;
b Exclusion criteria are including: ENT and eye inflammation, kidney, skin and peripheral nervous system involvement, lung infiltration, lymphadenopathies, stiff neck and digital gangrene or ulceration;
c No other aetiologies can better explain any one of the criteria;
d Enlarged and/or pulseless temporal artery: 1.p./tender temporal artery: 1.p;
e It must be ignored in the presence of PMR;
f Vascular and/or perivascular fibrinoid necrosis along with leucocyte infiltration: 1.p. /and granuloma: 1.p.

 

Proposal for expansion of the 1990 ACR criteria items for the classification of GCA

Original criteria Suggested expansion
Age at disease onset ≥50 years Age at disease onset ≥50 years
New onset headache of or new type of localized pain in the head Any of the following: New onset headache of new type of localized pain in the head, Visual symptoms, sight loss, PMR, Constitutional symptoms, Jaw and/or tongue claudication
Abnormality of temporal artery (tenderness to palpation or decreased pulsation unrelated to arteriosclerosis) Abnormality of temporal and/or extra-cranial arteries (tenderness to palpation or decreased pulsation, bruits of extra-cranial arteries unrelated to arteriosclerosis)
ESR ≥50 mm/h ESR ≥50 mm/h and/or CRP levels ≥10 mg/l
Abnormal artery biopsy Abnormal artery biopsy and/or abnormal imaging result (US, MRI and/or 18F-FDG PET)a

aThese parameters are opinion based and should not be used in clinical practice or in clinical studies. A patient shall be said to have GCA if three of the five criteria are present, as long as either temporal artery biopsy and/or imaging results are compatible with a diagnosis of GCA. 18F-FDG PET: 18-fluorine fluorodeoxyglucose PET/CT

 

References:

  1. Hunder GG, Bloch DA, Michel BA, Stevens MB, Arend WP, Calabrese LH, et al. The American College of Rheumatology 1990 criteria for the classification of giant cell arteritis. Arthritis Rheum. 1990;33:1122-8.[Medline]
  2. Weyand CM, Goronzy JJ. Clinical practice. Giant-cell arteritis and polymyalgia rheumatica. N Engl J Med. 2014 Jul 3;371(1):50-7. [Medline]
  3. Dejaco C, Duftner C, Buttgereit F, Matteson EL, Dasgupta B. The spectrum of giant cell arteritis and polymyalgia rheumatica: revisiting the concept of the disease. Rheumatology (Oxford). 2017 Apr 1;56(4):506-515. [Medline]
  4. Aghdam KA, Sanjari MS, Manafi N, Khorramdel S, Alemzadeh SA, Navahi RAA. Temporal Artery Biopsy for Diagnosing Giant Cell Arteritis: A Ten-year Review. J Ophthalmic Vis Res. 2020 Apr 6;15(2):201-209. [Medline]

 

Update: Jul 28, 2021

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