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title, docid, authors, breadcrumbs, category, documentVersionId, imageCount, lastUpdated, pageDescription, pageKeywords, pageTitle, enhancedTitle, type, references, breadcrumbs
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Giant Cell Arteritis 208eca17-81b8-448c-b8be-80e274dccc42
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ee6ece9d-ad74-458c-a8df-11628ae7f879 Arzu Canan, MD
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3d1e4c57-c1cf-4c89-b0f0-5d82b29a31e1 Suhny Abbara, MD, FACR, MSCCT, FNASCI
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10bb95ac-a27a-4ebe-833b-e59fea07734b Santiago Martínez-Jiménez, MD, FACR
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Giant Cell Arteritis giant-cell-arteritis null
Cardiac 9aabd711-33a6-4598-b5e2-495eff0adf14 16 11/14/24 Giant Cell Arteritis Cardiac, Diagnosis, Aorta, Giant Cell Arteritis Giant Cell Arteritis | STATdx Giant Cell Arteritis DX true
Cardiac
Diagnosis
Aorta
Giant Cell Arteritis

title: "Giant Cell Arteritis" docid: "208eca17-81b8-448c-b8be-80e274dccc42" authors:

  • key: "ee6ece9d-ad74-458c-a8df-11628ae7f879" value: "Arzu Canan, MD"
  • key: "3d1e4c57-c1cf-4c89-b0f0-5d82b29a31e1" value: "Suhny Abbara, MD, FACR, MSCCT, FNASCI"
  • key: "10bb95ac-a27a-4ebe-833b-e59fea07734b" value: "Santiago Mart\u00ednez-Jim\u00e9nez, MD, FACR" breadcrumbs:
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  • name: "Aorta" slug: "aorta" treeNodeId: "4d206a6b-1a82-467c-9199-0df25ab749d7"
  • name: "Giant Cell Arteritis" slug: "giant-cell-arteritis" treeNodeId: null category: "Cardiac" documentVersionId: "9aabd711-33a6-4598-b5e2-495eff0adf14" imageCount: 16 lastUpdated: "11/14/24" pageDescription: "Giant Cell Arteritis" pageKeywords: "Cardiac, Diagnosis, Aorta, Giant Cell Arteritis" pageTitle: "Giant Cell Arteritis | STATdx" enhancedTitle: "Giant Cell Arteritis" type: "DX" references: true breadcrumbs:
  • "Cardiac"
  • "Diagnosis"
  • "Aorta"
  • "Giant Cell Arteritis"

KEY FACTS

  • Terminology

    • Chronic, systemic, large or medium-sized, often granulomatous vasculitis - Often involves thoracic aorta and major branches - Often involves temporal artery

  • Imaging

    • CTA - Concentric aortic thickening (> 2 mm) - Aortic aneurysm; classically ascending aorta - Aortic dissection: Intimomedial flap
    • MR - Assessment of active inflammation - Delayed enhancement after gadolinium
    • Ultrasonography - High specificity and sensitivity; operator dependent - Hypoechoic halo temporal &/or axillary arteries
    • PET - Active inflammation demonstrates ↑ FDG uptake

  • Top Differential Diagnoses

    • Takayasu arteritis - May be identical to GCA - Extremely rare in patients > 50 years
    • Atherosclerotic disease - May be difficult to differentiate radiographically, though clinical symptoms often facilitate process - Similar age group

  • Clinical Issues

    • Headache, visual disturbances, jaw claudication
    • Polymyalgia rheumatica
    • Serologic markers - ↑ sedimentation rate - ↑ C-reactive protein - Thrombocytosis
    • Treatment - Corticosteroids

TERMINOLOGY

  • Abbreviations

    • Giant cell arteritis (GCA)

  • Synonyms

    • Temporal arteritis - Cranial GCA (C-GCA) often referred to as temporal arteritis; terminology not longer recommended, as sparing of temporal artery is not uncommon and because disease may involve large vessels - Horton disease

  • Definitions

    • Granulomatous autoimmune vasculitis affecting larger arteries and aorta - C-GCA: Often involves temporal artery and other head/neck vessels, but may also involve aorta and major branches - Large-vessel GCA (LV-GCA): Often involves thoracic aorta and major branches - Frequently associated with polymyalgia rheumatica (PMR) - Aching and morning stiffness in shoulders, hip girdle, and neck

IMAGING

  • General Features

    • Location

      - Temporal artery
- Aorta and aortic branches

  • CT Findings

    • NECT

      - Typically, GCA involving aorta is not as apparent or dense as intramural hematoma; however, there can be hyperdensity if associated with hemorrhage or calcification
- Transmural calcification is often similar to calcified atherosclerotic plaques (common)

    • CTA

      - Concentric aortic thickening (> 2 mm)
- Aortic stenosis
- Aortic aneurysm; classically ascending aorta
- Aortic dissection: Intimomedial flap
- Limited role in C-GCA

  • MR Findings

    • Equally accurate as CT for morphologic assessment on several sequences (e.g., T1WI, T2WI, HASTE, SSFP, etc.)
    • Contrast-enhanced MRA is more accurate to assess areas of stenosis and aneurysm
    • Assessment of active inflammation - Contrast-enhanced sequences: Delayed enhancement (i.e., ↑ signal) of vessel wall after gadolinium - Fat-saturated STIR sequence: High signal of thickened vessel wall
    • Cranial (temporal artery) involvement - High sensitivity and specificity - Mural thickening (> 0.5 mm) - Mural high T2 signal and contrast enhancement

  • Ultrasonographic Findings

    • Grayscale ultrasound

      - C-GCA
        - High specificity and sensitivity; operator dependent
        - Hypoechoic halo (i.e., **halo sign**) in temporal &/or axillary arteries
        - **Compression sign**: Persistence of halo during compression of vessel lumen by ultrasound probe

    • Color Doppler

      - Always in conjunction with grayscale ultrasound
- Helpful to localize temporal artery

  • Angiographic Findings

    • Stenosis (often long, regular, and smooth-walled)
    • Occlusion
    • Aneurysm
    • Limited in diagnosis of early vasculitis

  • Nuclear Medicine Findings

    • PET

      - LV-GCA: Active inflammation demonstrates ↑ FDG uptake
        - Subclinical inflammation of large vessels in 80% with GCA and ~ 30% PMR
        - Response to treatment correlates with ↓ FDG uptake
- Limited role in C-GCA, not recommended

  • Imaging Recommendations

    • Best imaging tool

      - MR
        - STIR: Thickening and high signal of aortic wall
        - Contrast-enhanced MR: Thickening and enhancement of aortic wall
        - MRA is helpful to detect areas of stenosis and aneurysm

    • Protocol advice

      - Consider concomitant NECT to differentiate from intramural hematoma
        - Caveat: GCA can occasionally be hyperdense

    • PET - Recognized role in patient with fever &/or inflammation of unknown origin - Unclear role in follow-up, especially asymptomatic patients without elevated inflammatory markers

DIFFERENTIAL DIAGNOSIS

  • Takayasu Arteritis

    • May have similar imaging appearance to GCA
    • Rare in patients > 50 years old

  • Other Systemic Vasculitides

    • e.g., polyarteritis nodosa, syphilitic aortitis
    • Occurs most often in small and medium-sized arteries
    • Biopsy and pattern of distribution often help differentiation

  • Fibromuscular Dysplasia

    • Most often affects renal arteries - Can also involve carotid arteries
    • Results in stenoses; occasional spontaneous dissection

  • Atherosclerotic Disease

    • May be difficult to differentiate radiographically, though clinical symptoms often facilitate process
    • Similar age group

PATHOLOGY

  • General Features

    • Etiology

      - Unknown
- Most accepted hypothesis: Antigen-driven disease mediated by T cells and macrophages that reach aortic wall via vasa vasorum

  • Staging, Grading, & Classification

    • Temporal artery biopsy remains diagnostic gold standard for C-GCA
    • Predictors of positive temporal artery biopsy - Jaw claudication - Neck pain - C-reactive protein > 2.45 mg/dL - Sedimentation rate > 47 mm/h - Thrombocytosis - Pallid optic disc edema - Temporal artery abnormalities
    • Temporal artery biopsy can be negative (10-15%)

  • Gross Pathologic & Surgical Features

    • Involvement of aorta (65.0%)
    • Involvement of main aortic tributaries (57.5%) - Brachiocephalic trunk (47.5%) - Subclavian arteries (42.5%) - Carotid arteries (35.0%) - Femoral arteries (30.0%) - Splanchnic arteries (22.5%) - Axillary arteries (17.5%) - Iliac arteries (15.0%) - Renal arteries (7.5%)

  • Microscopic Features

    • Focal chronic inflammatory cell infiltrates - Granulomas in vessel wall formed by CD4(+) T cells and macrophages
    • Focal areas of intimal hyperplasia - Proliferation of smooth muscle cells, which leads to narrowing of arterial lumen and eventually ischemia
    • Focal areas of fragmentation of inner elastic lamina
    • Focal concentric scars around inner elastic lamina

CLINICAL ISSUES

  • Presentation

    • Most common signs/symptoms

      - Headache
- Visual disturbances
- Jaw claudication

    • Other signs/symptoms

      - PMR
        - Present in 50% of patients at diagnosis of GCA
        - 20% of PMR will develop GCA
        - Clinical manifestations
                  - Morning stiffness
                  - Pain (shoulder > hip or neck)
                  - Synovitis and bursitis
                  - Swelling and tenosynovitis
                  - ↓ range of motion
                  - Muscle tenderness
                  - Subjective weakness
                  - Systemic signs and symptoms (e.g., malaise, fatigue, depression, anorexia, weight loss, fever)

    • Clinical profile

      - Clinical phenotypes
        - C-GCA (temporal arteritis with headache and visual disturbance)
        - LV-GCA (arm/limb claudication, chest pain)
        - PMR
        - Phenotypes can overlap
- Serologic markers
        - ↑ erythrocyte sedimentation rate
        - ↑ C-reactive protein
        - Thrombocytosis
- Association with HLA-DRB1*04
- LV-GCA linked to other systematic diseases, such as Behçet disease or hyper-IgG4 syndrome
- Factors for aneurysm formation
        - Aortic insufficiency
        - Murmur at time of diagnosis
        - Hyperlipemia
        - ↑ eritrosedimentation in combination with polymyalgia symptoms
        - ↑ levels of IL-2

  • Demographics

    • Age

      - Patients > 50 years old
- Incidence ↑ steadily with age

    • Sex

      - Women > men

    • Ethnicity

      - More common in people of Northern European and Scandinavian descent

    • Epidemiology

      - Prevalence in USA: 1 in 160,000
- Lifetime risk of developing GCA in USA: 1% in women and 0.5% in men

  • Natural History & Prognosis

    • Prognosis for visual recovery is poor
    • ↑ risk aortic aneurysm formation and dissection: 17-fold and 2.5x higher risk of thoracic and abdominal aortic aneurysms - ↓ survival rate
    • Involvement of coronary arteries may result in myocardial infarction or congestive heart failure
    • Bowel necrosis (uncommon)
    • 15-30% of PMR cases eventually develop GCA

  • Treatment

    • GCA and PMR: Corticosteroids
    • Aspirin
    • Other (2nd-line therapy) - Methotrexate - Azathioprine - Tocilizumab (IL-6 receptor alpha inhibitor)

DIAGNOSTIC CHECKLIST

  • Consider

    • Annual surveillance to assess for aneurysm and dissection
    • Alternatives for follow-up - Chest radiograph + echocardiogram + abdominal Doppler ultrasound - Contrast-enhanced CT of chest and abdomen

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References

Selected References

  1. Pepper K: Giant cell arteritis. Postgrad Med. 135(sup1):22-32, 2023
  2. Braun J et al: The role of 18F-FDG positron emission tomography for the diagnosis of vasculitides. Clin Exp Rheumatol. 36 Suppl 114(5):108-14, 2018
  3. Dejaco C et al: The spectrum of giant cell arteritis and polymyalgia rheumatica: revisiting the concept of the disease. Rheumatology (Oxford). 56(4):506-15, 2017
  4. Gomułka K et al: Horton's disease: still an important medical problem in elderly patients: a review and case report. Postepy Dermatol Alergol. 34(5):510-3, 2017
  5. Buttgereit F et al: Polymyalgia rheumatica and giant cell arteritis: a systematic review. JAMA. 315(22):2442-58, 2016
  6. Aschwanden M et al: The ultrasound compression sign to diagnose temporal giant cell arteritis shows an excellent interobserver agreement. Clin Exp Rheumatol. 33(2 Suppl 89):S-113-5, 2015
  7. Khan A et al: Imaging in giant cell arteritis. Curr Rheumatol Rep. 17(8):527, 2015
  8. Hartlage GR et al: Multimodality imaging of aortitis. JACC Cardiovasc Imaging. 7(6):605-19, 2014
  9. Schmidt WA: Ultrasound in vasculitis. Clin Exp Rheumatol. 32(1 Suppl 80):S71-7, 2014
  10. Jennette JC et al: 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum. 65(1):1-11, 2013
  11. Blockmans D: Diagnosis and extension of giant cell arteritis. Contribution of imaging techniques. Presse Med. 41(10):948-54, 2012
  12. Castañer E et al: Imaging findings in pulmonary vasculitis. Semin Ultrasound CT MR. 33(6):567-79, 2012
  13. Bossert M et al: Aortic involvement in giant cell arteritis: current data. Joint Bone Spine. 78(3):246-51, 2011
  14. Falardeau J: Giant cell arteritis. Neurol Clin. 28(3):581-91, 2010
  15. Bley TA et al: Diagnostic value of high-resolution MR imaging in giant cell arteritis. AJNR Am J Neuroradiol. 28(9):1722-7, 2007

Images

Selected Images

Axial CTA of the chest in a patient with giant cell arteritis (GCA) shows soft tissue density material surrounding the great vessels. (Courtesy C. S. Restrepo, MD.) Axial CTA of the chest in a patient with giant cell arteritis (GCA) shows soft tissue density material surrounding the great vessels. (Courtesy C. S. Restrepo, MD.)

Axial CTA of the chest in a patient with giant cell arteritis (GCA) shows soft tissue density material surrounding the great vessels. (Courtesy C. S. Restrepo, MD.) Axial CTA of the chest in a patient with giant cell arteritis (GCA) shows soft tissue density material surrounding the great vessels. (Courtesy C. S. Restrepo, MD.)

Axial chest CTA in the same patient shows concentric thickening of the thoracic aorta, which is a common finding in patients with GCA but is indistinguishable from Takayasu arteritis. GCA is more common in patients > 50 years old. Concomitant NECT is recommended to help differentiate from intramural hematoma. (Courtesy C. S. Restrepo, MD.) Axial chest CTA in the same patient shows concentric thickening of the thoracic aorta, which is a common finding in patients with GCA but is indistinguishable from Takayasu arteritis. GCA is more common in patients > 50 years old. Concomitant NECT is recommended to help differentiate from intramural hematoma. (Courtesy C. S. Restrepo, MD.)

Coronal FDG PET/CT in the same patient shows marked uptake of FDG along the ascending aortic wall . FDG PET has excellent sensitivity and specificity for the diagnosis of GCA and may be used when clinical or serological discrepancies arise during or after treatment of this condition. Coronal FDG PET/CT in the same patient shows marked uptake of FDG along the ascending aortic wall . FDG PET has excellent sensitivity and specificity for the diagnosis of GCA and may be used when clinical or serological discrepancies arise during or after treatment of this condition.

Coronal FDG PET/CT in a patient with GCA shows diffuse uptake along the ascending aortic wall as well as along the subclavian and axillary arteries bilaterally . Coronal FDG PET/CT in a patient with GCA shows diffuse uptake along the ascending aortic wall as well as along the subclavian and axillary arteries bilaterally .

Axial CTA in a patient with GCA shows diffuse arterial wall thickening and stranding of the periaortic fat. Note the reactive left pleural effusion . Axial CTA in a patient with GCA shows diffuse arterial wall thickening and stranding of the periaortic fat. Note the reactive left pleural effusion .

Axial double inversion recovery FS MR in the same patient at different levels shows diffuse high signal of the aortic wall as well as head and neck vessels . MR is the preferred method to assess for active inflammation also seen in the form of vessel parietal enhancement after intravenous gadolinium. Axial double inversion recovery FS MR in the same patient at different levels shows diffuse high signal of the aortic wall as well as head and neck vessels . MR is the preferred method to assess for active inflammation also seen in the form of vessel parietal enhancement after intravenous gadolinium.

Sagittal reformat CECT in a patient with GCA before and after contrast shows focal parietal thickening along the posterior descending thoracic aorta, only evident on CECT . Typically, vasculitis is not hyperdense on NECT as opposed to intramural hematoma. Sagittal reformat CECT in a patient with GCA before and after contrast shows focal parietal thickening along the posterior descending thoracic aorta, only evident on CECT . Typically, vasculitis is not hyperdense on NECT as opposed to intramural hematoma.

3D GRE MR (unenhanced and post contrast) at the same level shows progressive enhancement of the aortic wall after administration of intravenous contrast . 3D GRE MR (unenhanced and post contrast) at the same level shows progressive enhancement of the aortic wall after administration of intravenous contrast .

Axial CTA in a patient with unsuspected GCA who underwent reconstruction of the ascending aorta due to aneurysm is shown. Note the aneurysmal ascending and descending aorta . Axial CTA in a patient with unsuspected GCA who underwent reconstruction of the ascending aorta due to aneurysm is shown. Note the aneurysmal ascending and descending aorta .

Sagittal CECT MIP in the same patient shows diffuse aneurysmal thoracic aorta . Note also the aneurysmal right brachiocephalic trunk . Aneurysm is a very common complication of undiagnosed and untreated GCA only evident after resection. Sagittal CECT MIP in the same patient shows diffuse aneurysmal thoracic aorta . Note also the aneurysmal right brachiocephalic trunk . Aneurysm is a very common complication of undiagnosed and untreated GCA only evident after resection.

Additional Images

Axial CTA in a young patient shows mural thickening of the supraaortic great vessels and stranding of the adjacent perivascular fat due to a vasculitis. Note the stenosis of the left common carotid artery . Axial CTA in a young patient shows mural thickening of the supraaortic great vessels and stranding of the adjacent perivascular fat due to a vasculitis. Note the stenosis of the left common carotid artery .

Axial GRE MR following gadolinium administration shows marked circumferential mural thickening and enhancement of the descending thoracic aorta , consistent with active arteritis. Axial GRE MR following gadolinium administration shows marked circumferential mural thickening and enhancement of the descending thoracic aorta , consistent with active arteritis.

Axial GRE MR following gadolinium administration in the same patient confirms the presence of mural thickening and enhancement of the supraaortic arteries , consistent with active GCA. Axial GRE MR following gadolinium administration in the same patient confirms the presence of mural thickening and enhancement of the supraaortic arteries , consistent with active GCA.

Coronal contrast-enhanced MRA MIP in the same patient confirms multiple stenoses of the proximal pulmonary arteries without intraluminal thrombus. These are nonspecific features that are consistent with a pulmonary vasculitis, including GCA. Coronal contrast-enhanced MRA MIP in the same patient confirms multiple stenoses of the proximal pulmonary arteries without intraluminal thrombus. These are nonspecific features that are consistent with a pulmonary vasculitis, including GCA.

Axial CTA shows circumferential soft tissue thickening of the aortic arch in a patient with GCA. This represents an inflammatory reaction resulting in aortic mural thickening . Axial CTA shows circumferential soft tissue thickening of the aortic arch in a patient with GCA. This represents an inflammatory reaction resulting in aortic mural thickening .

Axial CTA shows irregular mural thickening of the descending thoracic aorta and pulmonary arteries . Mural thickening represents a common sequela of inflammatory arteritis. Axial CTA shows irregular mural thickening of the descending thoracic aorta and pulmonary arteries . Mural thickening represents a common sequela of inflammatory arteritis.

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