Difference between revisions of "Giant cell arteritis"

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Giant cell arteritis (view source)

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| Caption    = Giant cell arteritis. [[H&E stain]].
| Caption    = Giant cell arteritis. [[H&E stain]].
| Synonyms  = temporal arteritis
| Synonyms  = temporal arteritis
| Micro      = large artery with intramural inflammatory cells (often [[granuloma|granulomatous]]); destruction of arterial wall, i.e. fibrinoid necrosis (pink anucleate arterial wall)
| Micro      = large artery with intramural inflammatory cells (often [[granuloma|granulomatous]]); intimal thickening; frank destruction of arterial wall common - fibrinoid necrosis
| Subtypes  =
| Subtypes  =
| LMDDx      =
| LMDDx      =
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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      =
| Syndromes  =
| Syndromes  =
| Clinicalhx = patient older than 50 years
| Clinicalhx = typically older than 50 years
| Signs      = loss of vision, weight loss, chills, fever
| Signs      = loss of vision, weight loss, chills, fever
| Symptoms  = jaw claudication (classic), headache (classic), double vision, scalp tenderness
| Symptoms  = jaw claudication (classic), headache (classic), double vision, scalp tenderness
| Prevalence = uncommon
| Prevalence = uncommon
| Bloodwork  = ESR elevated
| Bloodwork  = ESR elevated
| Rads      =
| Rads      = halo sign
| Endoscopy  =
| Endoscopy  =
| Prognosis  = good if treated
| Prognosis  = good if treated
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Clinical features:
Clinical features:
*Classic finding: jaw claudication, in a patient older than 50 years.
*Classic finding: jaw claudication, typically in a patient older than 50 years.
*Other findings: headache (very common),<ref name=pmid25457237>{{Cite journal  | last1 = Le | first1 = K. | last2 = Bools | first2 = LM. | last3 = Lynn | first3 = AB. | last4 = Clancy | first4 = TV. | last5 = Hooks | first5 = WB. | last6 = Hope | first6 = WW. | title = The effect of temporal artery biopsy on the treatment of temporal arteritis. | journal = Am J Surg | volume =  | issue =  | pages =  | month = Oct | year = 2014 | doi = 10.1016/j.amjsurg.2014.07.007 | PMID = 25457237 }}</ref> vision loss or diplopia, scalp tenderness, polymyalgia, weight loss, chills, fever.
*Other findings: headache (very common),<ref name=pmid25457237>{{Cite journal  | last1 = Le | first1 = K. | last2 = Bools | first2 = LM. | last3 = Lynn | first3 = AB. | last4 = Clancy | first4 = TV. | last5 = Hooks | first5 = WB. | last6 = Hope | first6 = WW. | title = The effect of temporal artery biopsy on the treatment of temporal arteritis. | journal = Am J Surg | volume =  | issue =  | pages =  | month = Oct | year = 2014 | doi = 10.1016/j.amjsurg.2014.07.007 | PMID = 25457237 }}</ref> vision loss or diplopia, scalp tenderness, polymyalgia, weight loss, chills, fever.


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Treatment:
Treatment:
*Treat right away with high dose steroids.
*Treat right away with high dose steroids.
**Biopsy is confirmatory.
**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 intramural inflammatory cells.
*Artery with intimal thickening and luminal narrowing.
**Classically [[granuloma|granulomatous inflammation]].
*Transmural inflammatory cells.
***Granulomas not required for the diagnosis!
*Giant cells.
*Destruction of arterial wall, e.g. fibrinoid necrosis (pink anucleate arterial wall).
 
Notes:
*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).
*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===
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==Sign out==
==Sign out==
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>
===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===
<pre>
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.
</pre>
===Negative with atherosclerosis===
<pre>
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.
</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)
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.
</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>
===Negative===
===Negative===
<pre>
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.
</pre>
====Block letters====
<pre>
<pre>
TEMPORAL ARTERY, LEFT, BIOPSY:
TEMPORAL ARTERY, LEFT, BIOPSY:
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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 is
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>
Michael
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