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
Radiology halo sign
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]

Notes:

Microscopic

Features:[1][6]

  • Artery with intimal thickening and luminal narrowing.
  • Transmural inflammatory cells.
  • Giant cells.

Notes:

  • Inflammation classically granulomatous; however, granulomas not required for the diagnosis!
    • In one series, 11 of 15 patients (73%) had giant cells.[6]
    • In another series, 33 of 40 patients (83%) had giant cells on the initial biopsy.[7]
  • Often accompanied by frank destruction of the arterial wall, e.g. fibrinoid necrosis (pink anucleate arterial wall).
  • Luminal narrowing is common (>85% of cases) and typical marked.[6]

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)

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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.[8]


Positive with giant cells

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

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.

Alternate

Temporal Artery, Left, Biopsy:
     - Medium size artery with moderate-to-severe 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.

The sections show a focal histocytic response with intimal thickening. Giant cells are absent. Fibrinoid necrosis is absent. Significant transmural inflammation is absent.

Alternate

Temporal Artery, Right, Biopsy:
- Medium size artery with mild-to-moderate atherosclerosis, small calcifications and focal internal 
  elastic lamina disruption, 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. ↑ Nesher G, Shemesh D, Mates M, Sonnenblick M, Abramowitz HB (June 2002). "The predictive value of the halo sign in color Doppler ultrasonography of the temporal arteries for diagnosing giant cell arteritis". J Rheumatol 29 (6): 1224–6. PMID 12064840.
  6. ↑ 6.0 6.1 6.2 Roberts WC, Zafar S, Ko JM (April 2013). "Morphological features of temporal arteritis". Proc (Bayl Univ Med Cent) 26 (2): 109–15. doi:10.1080/08998280.2013.11928932. PMC 3603723. PMID 23543964. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3603723/.
  7. ↑ Maleszewski JJ, Younge BR, Fritzlen JT, Hunder GG, Goronzy JJ, Warrington KJ, Weyand CM (June 2017). "Clinical and pathological evolution of giant cell arteritis: a prospective study of follow-up temporal artery biopsies in 40 treated patients". Mod Pathol 30 (6): 788–796. doi:10.1038/modpathol.2017.10. PMC 5650068. PMID 28256573. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5650068/.
  8. ↑ 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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