Giant cell arteritis

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Giant cell arteritis (abbreviated GCA), also known as temporal arteritis, is a type of large vessel vasculitis.

Giant cell arteritis
Diagnosis in short

Giant cell arteritis. H&E stain.
Synonyms temporal arteritis
LM large artery with intramural inflammatory cells (often granulomatous); intimal thickening; frank destruction of arterial wall common - fibrinoid necrosis
Grossing notes temporal artery grossing
Site large blood vessels - see vasculitides
Clinical history typically older than 50 years
Signs loss of vision, weight loss, chills, fever
Symptoms jaw claudication (classic), headache (classic), double vision, scalp tenderness
Prevalence uncommon
Blood work ESR elevated
Prognosis good if treated
Clin. DDx other causes of headache
Treatment steroids

General

  • Classically afflicts the temporal artery.

Clinical features:

  • Classic finding: jaw claudication, typically in a patient older than 50 years.
  • Other findings: headache (very common),[1] vision loss or diplopia, scalp tenderness, polymyalgia, weight loss, chills, fever.

Work-up:

  • CRP, ESR, temporal artery biopsy.
    • ESR normal (>50 years old): <20 mm/hr males, <30 mm/hr females.[2]

Treatment:

  • Treat right away with high dose steroids.
    • Biopsy is confirmatory and is still diagnostic if done <7-10 days after treatment starts.[3]

Gross

  • Recommended length of artery >20 mm.[4]

Microscopic

Features - as per Le et al.:[1]

  • Artery with intimal thickening.
  • Transmural inflammatory cells.
  • Giant cells.

Notes:

  • Inflammation classically granulomatous.
    • Granulomas not required for the diagnosis!
  • Often accompanied by frank destruction of the arterial wall, e.g. fibrinoid necrosis (pink anucleate arterial wall).

DDx:

Images

  • GCA - very low mag. (WC)

  • GCA - low mag. (WC)

  • GCA - intermed. mag. (WC)

  • GCA - high mag. (WC)

  • GCA - intermed mag. (WC)

  • GCA - high mag. (WC)

www:

DDx:

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Note:

  • The evidence is weak that the biopsy result influences management; a negative biopsy doesn't preclude treatment for clinically presumed giant cell arteritis.[5]

Positive

Left Temporal Artery, Biopsy:
     - Consistent with temporal arteritis (medium size artery with 
       lymphohistocytic inflammation, arterial wall thickening, and 
       elastic fibre fragmentation).

Comment:
Giant cells are not seen. The findings should be correlated with the clinical impression.

Neutrophilic

Left Temporal Artery, Biopsy:
     - Consistent with temporal arteritis (medium size artery with 
       neutrophilic inflammation, fibrioid necrosis, and 
       elastic fibre fragmentation).

Comment:
Giant cells are not seen.

Minimal findings - positive

Left Temporal Artery, Biopsy:
     - Medium size artery with minimal lymphohistocytic inflammation 
       without definite giant cells or arterial wall thickening, see comment.

Comment:
The biopsy is suggestive of temporal arteritis that is either (1) early/poorly developed from a histomorphological perspective or (2) under-appreciated due to sampling.

The findings should be correlated with the clinical impression. The
management should be dependent upon the clinical impression.

Negative with atherosclerosis

Temporal Artery, Left, Biopsy:
- Medium size artery with mild-to-moderate atherosclerosis, otherwise 
  within normal limits, see comment.

Comment:
A negative biopsy does not rule out the possibility of giant cell (temporal) 
arteritis, as this may be a focal disorder. The clinical management should be
dependent upon the clinical impression.

Negative

Temporal Artery, Left, Biopsy:
- Medium size artery without pathologic diagnosis, see comment.

Comment:
A negative biopsy does not rule out the possibility of giant cell (temporal) 
arteritis, as this may be a focal disorder. The clinical management should be
dependent upon the clinical impression.

Block letters

TEMPORAL ARTERY, LEFT, BIOPSY:
- MEDIUM SIZE ARTERY WITHOUT PATHOLOGIC DIAGNOSIS, SEE COMMENT.

COMMENT:
A negative biopsy does not rule out the possibility of giant cell (temporal) 
arteritis, as this may be a focal disorder. The clinical management should be
dependent upon the clinical impression.

See also

References

  1. ↑ 1.0 1.1 Le, K.; Bools, LM.; Lynn, AB.; Clancy, TV.; Hooks, WB.; Hope, WW. (Oct 2014). "The effect of temporal artery biopsy on the treatment of temporal arteritis.". Am J Surg. doi:10.1016/j.amjsurg.2014.07.007. PMID 25457237.
  2. ↑ URL: http://www.nlm.nih.gov/medlineplus/ency/article/003638.htm. Accessed on: 17 August 2012.
  3. ↑ Weinberg, DA.; Savino, PJ.; Sergott, RC.; Bosley, TM. (Jul 1994). "Giant cell arteritis. Corticosteroids, temporal artery biopsy, and blindness.". Arch Fam Med 3 (7): 623-7. PMID 7921300.
  4. ↑ Sharma, NS.; Ooi, JL.; McGarity, BH.; Vollmer-Conna, U.; McCluskey, P. (Jun 2007). "The length of superficial temporal artery biopsies.". ANZ J Surg 77 (6): 437-9. doi:10.1111/j.1445-2197.2007.04090.x. PMID 17501882.
  5. ↑ Lenton, J.; Donnelly, R.; Nash, JR. (Jan 2006). "Does temporal artery biopsy influence the management of temporal arteritis?". QJM 99 (1): 33-6. doi:10.1093/qjmed/hci141. PMID 16287908.
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