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Temporal arteritis

Authors: José Tomás Peña, M.D., Pablo Zoroquiain, M.D.

Last author update: 8 December 2022

Last staff update: 28 April 2023 (update in progress)

Copyright: 2004-2023, PathologyOutlines.com, Inc.

PubMed Search: Temporal arteritis

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Table of Contents

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

Terminology

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

Epidemiology

Most common systemic / primary vasculitis (Semin Arthritis Rheum 2020;50:1040)
Age: > 50 years; mean: 70 years; peak: 71 - 80 years
Gender: F:M = 3:1
Race / ethnicity: more common in Caucasians; rare in Asian and African American patients
Incidence (ACR classification criteria):
- Northern Europe (Norway): 29 - 32.8 / 100,000 (J Rheumatol 1997;24:1739, J Rheumatol 2000;27:2624)
- U.S. (Minnesota): 19.8 / 100,000 (Scand J Rheumatol 2015;44:215)
- Southern Europe (Spain): 2.2 - 10.2 / 100,000 (Clin Exp Rheumatol 2015;33:S-11, Rheum Dis Clin North Am 2001;27:729)
Seasonal variation: there is no seasonal influence (RMD Open 2017;3:e000531)
Mortality (Rheumatology (Oxford) 2022;61:1195):
- All cause mortality:
  - 6.4% at 1 year (relative risk 1.49 ± 0.13); 45% at 10 years (relative risk 1.03 ± 0.02)
- Cause specific mortality:
  - Compared to the general population, there is increased mortality due to infectious, endocrine, cardiovascular and gastrointestinal diseases
  - This increased mortality may be corticosteroid related (J Rheumatol 2017;44:84)

Sites

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

Pathophysiology

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

Etiology

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)
- Can be elicited by the chewing gum test (N Engl J Med 2016;374:1794)
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

Diagnosis requires morphologic confirmation
- EULAR 2018: diagnostic confirmation can be performed by imaging or histology (Ann Rheum Dis 2020;79:19)
- ACR / Vasculitis Foundation 2021: temporal artery biopsy is the preferred modality for diagnostic confirmation (Arthritis Rheumatol 2021;73:1349)
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)
- Optimal length of biopsy: at least 1.5 cm (ANZ J Surg 2018;88:191)
- Unilateral temporal artery biopsy is the preferred modality
- Bilateral temporal artery biopsy: increases diagnostic yield up to 12% (J Vasc Surg 2022;76:1704)

Laboratory

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:

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

68 year old woman with tongue necrosis (Case Rep Med 2017;2017:6327437)
71 year old woman with headache and normal inflammatory markers (Clin Med (Lond) 2020;20:224)
72 year old man with an aortic aneurysm (Radiol Case Rep 2021;16:3734)
79 year old man with vision loss (Rheumatol Int 2022;42:1855)
81 year old woman with scalp ulceration (BMJ Case Rep 2019;12:e230795)

Treatment

Corticosteroids as soon as possible
Can be combined with corticosteroid sparing drugs

Clinical images

Images hosted on other servers:

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

Loss of elastic fibers

Virtual slides

Images hosted on other servers:

Giant cell arteritis in temporal artery

Giant cell arteritis with intimal hyperplasia

Positive stains

Elastic stain, CD68, CD3 (Am J Surg Pathol 2014;38:1360)

Videos

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

Additional references

Burke: Practical Cardiovascular Pathology, 1st Edition, 2010, Salvarani: Large and Medium Size Vessel and Single Organ Vasculitis, 1st Edition, 2021

Board review style question #1

Which of the following antibody panels is most useful for evaluating temporal arteritis?

CD3 / CD68
CD4 / CD8
CD20 / CD138
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?

Intimal hyperplasia
Loss of elastic fibers
Myxoid degeneration
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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