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Difference between revisions of "Giant cell arteritis"
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| IF = | | IF = | ||
| Gross = | | Gross = | ||
| Grossing = | | Grossing = [[temporal artery grossing]] | ||
| Site = large [[blood vessels]] - see ''[[vasculitides]]'' | | Site = large [[blood vessels]] - see ''[[vasculitides]]'' | ||
| Assdx = | | Assdx = | ||
| Line 24: | Line 24: | ||
| Prevalence = uncommon | | Prevalence = uncommon | ||
| Bloodwork = ESR elevated | | Bloodwork = ESR elevated | ||
| Rads = | | Rads = halo sign | ||
| Endoscopy = | | Endoscopy = | ||
| Prognosis = good if treated | | Prognosis = good if treated | ||
| Line 47: | Line 47: | ||
*Treat right away with high dose steroids. | *Treat right away with high dose steroids. | ||
**Biopsy is confirmatory and is still diagnostic if done <7-10 days after treatment starts.<ref name=pmid7921300>{{Cite journal | last1 = Weinberg | first1 = DA. | last2 = Savino | first2 = PJ. | last3 = Sergott | first3 = RC. | last4 = Bosley | first4 = TM. | title = Giant cell arteritis. Corticosteroids, temporal artery biopsy, and blindness. | journal = Arch Fam Med | volume = 3 | issue = 7 | pages = 623-7 | month = Jul | year = 1994 | doi = | PMID = 7921300 }}</ref> | **Biopsy is confirmatory and is still diagnostic if done <7-10 days after treatment starts.<ref name=pmid7921300>{{Cite journal | last1 = Weinberg | first1 = DA. | last2 = Savino | first2 = PJ. | last3 = Sergott | first3 = RC. | last4 = Bosley | first4 = TM. | title = Giant cell arteritis. Corticosteroids, temporal artery biopsy, and blindness. | journal = Arch Fam Med | volume = 3 | issue = 7 | pages = 623-7 | month = Jul | year = 1994 | doi = | PMID = 7921300 }}</ref> | ||
==Gross== | |||
*Recommended length of artery >20 mm.<ref name=pmid17501882>{{Cite journal | last1 = Sharma | first1 = NS. | last2 = Ooi | first2 = JL. | last3 = McGarity | first3 = BH. | last4 = Vollmer-Conna | first4 = U. | last5 = McCluskey | first5 = P. | title = The length of superficial temporal artery biopsies. | journal = ANZ J Surg | volume = 77 | issue = 6 | pages = 437-9 | month = Jun | year = 2007 | doi = 10.1111/j.1445-2197.2007.04090.x | PMID = 17501882 }}</ref> | |||
Notes: | |||
*Radiology: halo sign (on ultrasound); [[sensitivity]] 86% and [[specificity]] 78%.<ref name=pmid12064840>{{cite journal |authors=Nesher G, Shemesh D, Mates M, Sonnenblick M, Abramowitz HB |title=The predictive value of the halo sign in color Doppler ultrasonography of the temporal arteries for diagnosing giant cell arteritis |journal=J Rheumatol |volume=29 |issue=6 |pages=1224–6 |date=June 2002 |pmid=12064840 |doi= |url=}}</ref> | |||
==Microscopic== | ==Microscopic== | ||
Features | Features:<ref name=pmid25457237/><ref name=pmid23543964/> | ||
*Artery with intimal thickening. | *Artery with intimal thickening and luminal narrowing. | ||
*Transmural inflammatory cells. | *Transmural inflammatory cells. | ||
*Giant cells. | *Giant cells. | ||
Notes: | Notes: | ||
*Inflammation classically [[granuloma|granulomatous]] | *Inflammation classically [[granuloma|granulomatous]]; however, granulomas not required for the diagnosis! | ||
**In one series, 11 of 15 patients (73%) had giant cells.<ref name=pmid23543964>{{cite journal |authors=Roberts WC, Zafar S, Ko JM |title=Morphological features of temporal arteritis |journal=Proc (Bayl Univ Med Cent) |volume=26 |issue=2 |pages=109–15 |date=April 2013 |pmid=23543964 |pmc=3603723 |doi=10.1080/08998280.2013.11928932 |url=}}</ref> | |||
**In another series, 33 of 40 patients (83%) had giant cells on the initial biopsy.<ref name=pmid28256573>{{cite journal |authors=Maleszewski JJ, Younge BR, Fritzlen JT, Hunder GG, Goronzy JJ, Warrington KJ, Weyand CM |title=Clinical and pathological evolution of giant cell arteritis: a prospective study of follow-up temporal artery biopsies in 40 treated patients |journal=Mod Pathol |volume=30 |issue=6 |pages=788–796 |date=June 2017 |pmid=28256573 |pmc=5650068 |doi=10.1038/modpathol.2017.10 |url=}}</ref> | |||
*Often accompanied by frank destruction of the arterial wall, e.g. fibrinoid necrosis (pink anucleate arterial wall). | *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.<ref name=pmid23543964/> | |||
DDx: | |||
* [[Atherosclerosis]]. | |||
* [[Vasculitides#Takayasu_arteritis|Takayasu arteritis]] - can be overlapping with GCA. | |||
* [[Aneurysm]]. | |||
* [[Amyloidosis]]. | |||
* [[Granulomatosis with polyangiitis]] - Wegener Granulomatosis. | |||
* [[Polyarteritis nodosa]]. | |||
===Images=== | ===Images=== | ||
| Line 71: | Line 87: | ||
*[http://www.djo.harvard.edu/files/5077_728.jpg GCA (harvard.edu)]. | *[http://www.djo.harvard.edu/files/5077_728.jpg GCA (harvard.edu)]. | ||
*[http://path.upmc.edu/cases/case646.html GCA - several images (upmc.edu)]. | *[http://path.upmc.edu/cases/case646.html GCA - several images (upmc.edu)]. | ||
==Sign out== | ==Sign out== | ||
Note: | Note: | ||
*The evidence is weak that the biopsy result influences management; a negative biopsy doesn't preclude treatment for clinically presumed giant cell arteritis.<ref name=pmid16287908>{{Cite journal | last1 = Lenton | first1 = J. | last2 = Donnelly | first2 = R. | last3 = Nash | first3 = JR. | title = Does temporal artery biopsy influence the management of temporal arteritis? | journal = QJM | volume = 99 | issue = 1 | pages = 33-6 | month = Jan | year = 2006 | doi = 10.1093/qjmed/hci141 | PMID = 16287908 }}</ref> | *The evidence is weak that the biopsy result influences management; a negative biopsy doesn't preclude treatment for clinically presumed giant cell arteritis.<ref name=pmid16287908>{{Cite journal | last1 = Lenton | first1 = J. | last2 = Donnelly | first2 = R. | last3 = Nash | first3 = JR. | title = Does temporal artery biopsy influence the management of temporal arteritis? | journal = QJM | volume = 99 | issue = 1 | pages = 33-6 | month = Jan | year = 2006 | doi = 10.1093/qjmed/hci141 | PMID = 16287908 }}</ref> | ||
===Positive with giant cells=== | |||
<pre> | |||
Left Temporal Artery, Biopsy: | |||
- Consistent with temporal arteritis (medium size artery with | |||
lymphohistocytic inflammation, giant cells, arterial wall thickening, | |||
and elastic fibre fragmentation). | |||
</pre> | |||
===Positive=== | |||
<pre> | |||
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. | |||
</pre> | |||
====Neutrophilic==== | |||
<pre> | |||
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. | |||
</pre> | |||
===Minimal findings - positive=== | ===Minimal findings - positive=== | ||
<pre> | <pre> | ||
Left Temporal Artery, Biopsy: | Left Temporal Artery, Biopsy: | ||
- Medium size artery with | - 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 | The findings should be correlated with the clinical impression. The | ||
management should be dependent upon the clinical impression. | management should be dependent upon the clinical impression. | ||
| Line 100: | Line 142: | ||
within normal limits, see comment. | 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. | |||
</pre> | |||
====Alternate==== | |||
<pre> | |||
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) | A negative biopsy does not rule out the possibility of giant cell (temporal) | ||
arteritis, as this may be a focal disorder. The clinical management | arteritis, as this may be a focal disorder. The clinical management should be | ||
dependent upon the clinical impression. | 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. | |||
</pre> | |||
====Alternate==== | |||
<pre> | |||
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. | |||
</pre> | </pre> | ||
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- Medium size artery without pathologic diagnosis, see comment. | - Medium size artery without pathologic diagnosis, see comment. | ||
Comment: | |||
A negative biopsy does not rule out the possibility of giant cell (temporal) | A negative biopsy does not rule out the possibility of giant cell (temporal) | ||
arteritis, as this may be a focal disorder. The clinical management | arteritis, as this may be a focal disorder. The clinical management should be | ||
dependent upon the clinical impression. | dependent upon the clinical impression. | ||
</pre> | </pre> | ||
| Line 124: | Line 190: | ||
COMMENT: | COMMENT: | ||
A negative biopsy does not rule out the possibility of giant cell (temporal) | A negative biopsy does not rule out the possibility of giant cell (temporal) | ||
arteritis, as this may be a focal disorder. The clinical management | arteritis, as this may be a focal disorder. The clinical management should be | ||
dependent upon the clinical impression. | dependent upon the clinical impression. | ||
</pre> | </pre> | ||