AJR 2003; 181:742
© American Roentgen Ray Society
Radiologic-Pathologic Conference of Wilford Hall Medical
Center |
Giant Cell Arteritis
Jennifer L. Bau1,2,
Justin Q. Ly1,
Gregory C. Borstad3,
Joanna D. Lusk4,
Thomas M. Seay1 and
Douglas P. Beall1,5
1 Department of Radiology, Wilford Hall Medical Center, Ste. 1, 2200 Bergquist
Dr., Lackland AFB, TX 78236-5300.
2 Pennsylvania State University College of Medicine, Hershey Medical Center, 500
University Dr., Hershey, PA 17033.
3 Department of Rheumatology, Wilford Hall Medical Center, Lackland AFB, TX
78236-5300.
4 Department of Pathology, Wilford Hall Medical Center, Lackland AFB, TX
78236-5300.
Received December 2, 2002;
accepted after revision January 13, 2003.
The opinions and assertions contained herein are the private views of the
authors and are not to be construed as official or as representing the views
of the Department of the Air Force or the Department of Defense.
Address correspondence to J. Q. Ly.
5 Present address: Department of Radiology, Uniformed Services University of
the Health Sciences, 4301 Jones Bridge Rd., Bethesda, MD 20814-4799.
A64-year-old woman presented with fever, chills, night sweats, fatigue,
mild dyspnea on exertion, and an elevated erythrocyte sedimentation rate. A
positive result from a purified protein derivative test prompted further
thoracic evaluation with contrast-enhanced chest CT, which revealed diffuse
thickening of the walls of the entire thoracic aorta and proximal great
vessels (Figs. 1A,
1B,
1C), findings suspicious for
vasculitis. These findings, in association with the patient's reported recent
onset of bifrontal headaches and jaw claudication, prompted a temporal artery
biopsy. The final histologic diagnosis was giant cell arteritis
(Fig. 1D).
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Fig. 1A. —64-year-old woman with giant cell arteritis involving great
vessels. Axial contrast-enhanced chest CT image shows diffuse thickening
(arrowheads) of walls of great vessels (left to right: right
brachiocephalic artery, left common carotid artery, left subclavian
artery).
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Fig. 1D. —64-year-old woman with giant cell arteritis involving great
vessels. Photomicrograph of biopsied artery shows granulomatous inflammation
of inner half of media (arrows) with disruption of internal elastic
lamina. (H and E, x40)
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Giant cell arteritis, or temporal arteritis, is one of the most common
forms of vasculitis in the United States and northern Europe. Occurring more
often in women and in individuals over the age of 50 years, this pathologic
condition affects the primary and secondary branches of the aorta and
sometimes the aorta itself [1,
2]. Patients may present with
fatigue, fever, weight loss, vision loss, headache, arm or jaw claudication,
and scalp tenderness. A markedly elevated erythrocyte sedimentation rate is
almost always present. Little is known about the incidence or the nature of
aortitis in the setting of giant cell arteritis, although it is widely
accepted that aortic aneurysm and dissection are more common in these patients
[3]. Several theories exist as
to its pathogenesis, with many authors theorizing that giant cell arteritis is
an immune response associated with genetic predisposition or that it is caused
by an infectious agent [1,
2].
Histologically, giant cell arteritis appears similar to Takayasu's
arteritis, showing granulomatous inflammation of arteries with infiltration
predominantly by histiocytes, lymphocytes, and multinucleated giant cells.
Multinucleated giant cells are identified, however, in only 50% of temporal
artery biopsies in cases of giant cell arteritis; therefore this histologic
finding is not required for diagnosis. Moreover, the most active inflammation
characteristically occurs along the internal elastic lamina of involved
vessels [1].
Temporal arteriography may reveal the extent of vessels involved but is
generally invasive and not sufficient for detecting milder degrees of
vasculitis [2]. CT angiography
is useful for revealing luminal changes, such as stenosis, occlusion,
dilation, and aneurysm. It is also useful for showing mural changes to include
wall thickening, which correlate well with the inflammatory changes seen
histologically, as was the case in this patient. CT may also show
calcification and mural thrombi, which may not be adequately visualized on
conventional angiography. CT angiography is reportedly 95% sensitive and 100%
specific for diagnosing Takayasu's arteritis
[4], and similar results may be
expected for patients with giant cell arteritis.
Prompt diagnosis and treatment of giant cell arteritis are important to
reduce patient morbidity. In particular, vision loss can last for several
hours or may be permanent; as many as 15-20% of patients with giant cell
arteritis have permanent loss of vision
[1].
References
- Hunder G. Vasculitis: diagnosis and therapy. Am J
Med 1996;100[suppl]:37S
-45S[Medline]
- Hunder GG, Weyand CM. Sonography in giant cell arteritis.
N Engl J Med1997; 337:1385
-1386
- Evans JE, Hunder GG. The implications of recognizing large-vessel
involvement in elderly patients with giant cell arteritis. Curr
Opin Rheumatol 1997;9:37
-40[Medline]
- Yamada I, Nakagawa T, Himeno Y, Numano F, Shibuya H. Takayasu
arteritis: evaluation of the thoracic aorta with CT angiography.
Radiology1998; 209:103
-109[Abstract/Free Full Text]
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