Orbit & optic nerve

Temporal arteritis

Last author update: 8 December 2022
Last staff update: 28 April 2023 (update in progress)

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PubMed Search: Temporal arteritis

José Tomás Peña, M.D.
Pablo Zoroquiain, M.D.
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Cite this page: Peña JT, Zoroquiain P. Temporal arteritis. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/eyeorbittemporalarteritis.html. Accessed October 2nd, 2023.
Definition / general
  • Giant cell arteritis is a systemic and discontinuous nonnecrotizing vasculitis of elastic and muscular arteries
Essential features
  • Presents in patients in the sixth decade of life or older with new onset headache
  • Risk of permanent vision loss if left untreated
  • Diagnosis requires morphologic confirmation (biopsy is the preferred modality)
  • Classic findings include lymphocytes and macrophages arranged in concentric rings surrounding the external and internal elastic lamina as well as an association with intimal hyperplasia and loss / fragmentation of elastic fibers
  • Useful stains: elastic stain, CD3 and CD68
  • Temporal arteritis
  • Giant cell arteritis
  • Horton disease
  • Horton giant cell arteritis
  • Cranial arteritis
ICD coding
  • ICD-10:
    • M31.5 - giant cell arteritis with polymyalgia rheumatica
    • M31.6 - other giant cell arteritis
  • Cranial arteries: temporal artery, occipital artery and intraorbital branches (ophthalmic and posterior ciliary)
  • Extracranial arteries are compromised in 20 - 67% of cases (angiography) (Arthritis Care Res (Hoboken) 2020;72:1615)
    • Most common sites: carotid, subclavian, axillary and thoracic aorta
    • Bilateral axillary artery compromise is common
  • Immunopathological model as described by Greigert et al. (J Clin Med 2022;11:2905):
    • Phase 1: loss of tolerance and activation of the adventitia resident dendritic cells
      • Dendritic cells acquire mature phenotype and produce IL12, IL23, IL6 and IL1β
    • Phase 2: recruitment, activation and polarization of CD4+ T cells
      • CD4+ T cells polarize to Th1 and Th17 phenotypes
    • Phase 3: recruitment of CD8+ T cells and monocytes
    • Phase 4: vascular remodeling
      • Macrophages release several factors, such as reactive oxygen species and matrix metalloproteinases, which mediate internal elastic lamina destruction
      • Vascular smooth muscle cells migrate to the intima and acquire a myofibroblast phenotype
      • Myofibroblasts produce intimal hyperplasia, which then causes vessel occlusion and subsequent ischemia
  • Immune mediated disease of unknown etiology
  • Postulated genetic predisposing factors (Sci Rep 2017;7:43953):
    • Major histocompatibility class II genes (HLA-DRB1*04 alleles)
    • IL12B locus
Clinical features
  • Headache (73%)
  • Tender temporal artery (31%)
  • Scalp tenderness (44%)
  • Jaw claudication (45%) (best predictor of positive biopsy)
  • Visual disturbances:
    • Blurred vision (18%)
    • Transient vision loss (5%)
    • Permanent vision loss (6%), due to ischemic optic neuropathy
  • Fever (16%)
  • Weight loss (25%)
  • 25% of patients can be asymptomatic
  • Comorbidities:
    • Polymyalgia rheumatica (PMR) (Rheumatology (Oxford) 2017;56:506):
      • 40 - 60% of patients with giant cell arteritis have signs and symptoms of polymyalgia rheumatica
      • 16 - 21% of patients with polymyalgia rheumatica develop giant cell arteritis
    • Symptomatic thoracic aneurysm: 10 - 15% of giant cell arteritis patients develop symptomatic thoracic aneurysms
  • Reference: Semin Arthritis Rheum 2021;51:1193
  • Diagnosis requires morphologic confirmation
  • Ultrasonography: offers good sensitivity and specificity in treatment naive patients (up to 4 days of treatment)
  • Temporal artery biopsy (TAB): offers good sensitivity and specificity within 2 - 4 weeks of glucocorticoid treatment (BMC Musculoskelet Disord 2016;17:363)
  • Elevated erythrocyte sedimentation rate
  • Elevated C reactive protein
Radiology description
  • High frequency Doppler ultrasound:
    • Halo sign (sensitivity 80%, specificity 80%): hypoechoic halo surrounding temporal artery lumen (J Ultrasound 2022;25:837)
    • Compression sign (sensitivity 85%, specificity 65%): compression of the temporal artery produces contrasting echogenicity in the surrounding tissue (Clin Exp Rheumatol 2015;33:S-113)
Radiology images

Images hosted on other servers:
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Halo sign

Prognostic factors
  • Predictors associated with reduced risk of permanent visual loss:
    • Fever
    • Rheumatic symptoms
  • Predictors associated with increased risk of permanent visual loss:
    • Age
    • History of transient visual ischemic symptoms
    • Jaw claudication
  • Reference: J Rheumatol 2016;43:1393
Case reports
  • Corticosteroids as soon as possible
  • Can be combined with corticosteroid sparing drugs
Clinical images

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Visible temporal artery

Gross description
  • Segment of muscular artery with thickened and rubbery wall
Microscopic (histologic) description
  • Classic giant cell arteritis (77% of cases) (Am J Surg Pathol 2014;38:1360):
    • Transmural inflammation pattern with lymphocytes and macrophages arranged in concentric rings, surrounding the external and internal elastic lamina and associated with intimal hyperplasia and loss of elastic fibers
      • 50 - 70% of cases exhibit giant cells
      • Neutrophils are uncommon (1.8%)
    • Necrosis, well formed granulomas and thrombosis are uncommon
  • Borderline arteritis - lymphocytic and histiocytic infiltrate restricted to the following localizations:
    • Vasa vasorum vasculitis (6.5% of cases): inflammation of vasa vasorum in the absence of medial inflammation
    • Inflammation limited to the adventitia (7% of cases): crescent shaped configuration limited to the adventitia and external elastic lamina, with sparing of the media
    • Small vessel vasculitis (9% of cases): inflammatory lymphocytic infiltrate surrounding (cuffing) small periadventitial vessels
  • Healed arteritis - fibrotic changes without inflammatory lymphocytic infiltrate:
    • Marked intimal thickening
    • Intimal, medial and adventitial fibrosis and scarring, with neovascularization
    • Loss of elastic fibers
Microscopic (histologic) images

Contributed by José Tomás Peña, M.D., Pablo Zoroquiain, M.D. and @MirunaPopescu13 on Twitter

Segmental inflammation

Intimal hyperplasia

Transmural inflammation

Giant cells

Temporal arteritis

Temporal arteritis

Loss of elastic fibers

Loss of elastic fibers

Virtual slides

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Giant cell arteritis in temporal artery

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Giant cell arteritis with intimal hyperplasia


Vasculitis Foundation giant cell arteritis webinar

Giant cell arteritis
by Nicholas J. Volpe,
European Society of Ophthalmology

Temporal artery biopsy technique

Sample pathology report
  • Right temporal artery, biopsy:
    • Morphological findings consistent with temporal arteritis (see comment)
    • Comment: There is lymphocytic and macrophagic infiltration within the vascular wall. Multinucleated giant cell phagocytizing elastic fibers (elastic stain) are observed, supporting the diagnosis.
Differential diagnosis
  • Age related changes:
    • Intimal hyperplasia, duplication of the elastic inner lamina, adventitial fibrosis
    • Absence of mononuclear infiltration of the arterial wall
  • Another vasculitis (up to 5% of cases):
    • Neutrophil infiltration, abundant fibrinoid necrosis, microthrombi and leukocytoclasis
  • AL amyloidosis (< 1%) (Mod Rheumatol Case Rep 2020;4:90):
    • Intramural amorphous eosinophilic material deposits
    • Light chain immunofixation in urine
    • Congo red+ with green dichroism
  • Calciphylaxis (< 1%) (J Neuroophthalmol 2022;42:e362):
    • Calcium deposition in vessels
    • Numerous thrombi
    • Necrosis of distal territories
  • Atherosclerosis (< 1%) (J Neuroophthalmol 2022;42:e359):
    • Intimal hyperplasia, foamy macrophages (early)
    • Intimal fibrosis, cholesterol cleft and variable inflammatory cell infiltration within the intima (chronic)
    • No medial inflammation, no phagocytosis of elastic fibers
  • Mönckeberg medial calcific sclerosis of the temporal artery (< 1%) (Cureus 2020;12:e9210):
    • Annular calcification within media
    • No thrombus
    • No inflammatory infiltrate
Board review style question #1
Which of the following antibody panels is most useful for evaluating temporal arteritis?

  1. CD3 / CD68
  2. CD4 / CD8
  3. CD20 / CD138
  4. CD34 / CD68
Board review style answer #1
A. CD3 / CD68. The hallmark of temporal arteritis is a transmural lymphocytic (T cells) and macrophagic infiltrate, which can be demonstrated with antibodies against CD3 and CD68, respectively. B cells (CD20+) and plasma cells (CD138+) are not diagnostic findings. The combination of CD34 and CD68 highlights histiocytes, myofibroblasts and small blood vessels but excludes T cells. Although CD4+ and CD8+ are T cell markers, this panel does not stain macrophages.

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Reference: Temporal arteritis
Board review style question #2

A patient is diagnosed with temporal arteritis. What key finding is demonstrated with the special stain used in the image above?

  1. Intimal hyperplasia
  2. Loss of elastic fibers
  3. Myxoid degeneration
  4. Tunica media fibrosis
Board review style answer #2
B. Loss of elastic fibers. The Verhoeff-Van Gieson stain is a special stain used to demonstrate elastic fibers. In the setting of temporal arteritis, it can be used to evaluate areas of loss and disorganization of elastic fibers. Intimal hyperplasia is visible with H&E and does not require special stains. Tunica media fibrosis is a nonspecific finding and can be observed in the scenario of healed arteritis. Myxoid degeneration is not a characteristic finding of temporal arteritis.

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Reference: Temporal arteritis
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