DOI: 10.1148/radiol.2272010487
(Radiology 2003;227:512-515.)
© RSNA, 2003
Case 58: Giant Cell Arteritis1
Mark E. Lockhart, MD and
Michelle L. Robbin, MD
1 From the Department of Radiology, University of Alabama at Birmingham, 619 19th St South, Rm JTN358, Birmingham, AL 35249-6830. Received February 19, 2001; revision requested April 3; revision received November 26; accepted January 7, 2002. Address correspondence to M.E.L. (e-mail: mlockhar@uabmc.edu).
Index terms: Arteries, US, 955.12983 • Arteritis, 955.7229 • Diagnosis Please • Gastrointestinal tract, vasculitis, 955.62, 955.7229 • Ultrasound (US), Doppler studies, 955.12983 • Vasculitis, 955.62, 955.7229
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HISTORY
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A 72-year-old black woman had a history of abdominal pain, diarrhea, and weight loss of approximately 4 months duration at presentation to her primary care physician. The pain was initially episodic and did not localize to a specific quadrant. In the 4 weeks prior to presentation, cramps and abdominal pain were associated with meals and were relieved after a bowel movement. She did not have a fever or the chills. Her medical history included recurrent right-sided headaches, partial loss of vision in the right eye, anemia, and peripheral vascular disease for which she underwent bypass graft surgery. At physical examination, there was no marked abdominal tenderness and there were no peritoneal signs of disease. Bowel sounds were normal. Laboratory values were normal, and tests for rheumatoid factor and antinuclear antibodies were negative.
Findings of an initial small bowel series did not suggest any abnormalities. The patient had an abnormal angiogram that was obtained at another institution, and findings reportedly suggested diffuse arterial abnormalities (ie, abnormalities of the temporal, iliac, renal, and mesenteric arteries). The patient had worsening signs of bowel ischemia, and bypass surgery was performed to treat a distal superior mesenteric artery (SMA) occlusion. Intraoperative color and spectral Doppler ultrasonography (US) was requested to examine the aorta and visceral vessels for evidence of stenosis or occlusion during bypass surgery performed in the SMA. Transverse (Fig 1a) and longitudinal (Fig 1b) images of the SMA are shown. Color and spectral Doppler US was performed to characterize the degree and extent of SMA stenosis. Visualization of an optimal nonstenotic segment of SMA for bypass surgery was desired. Examination of the celiac artery did not disclose any abnormalities. The inferior mesenteric artery was not examined with US.
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Figure 1a. Representative intraoperative (a) transverse and (b) longitudinal color Doppler US images of the SMA. Diffuse symmetric hypoechoic halo (cursors) surrounds the patent lumen. The vessel is narrowed and is 1.3-1.7 mm in diameter. (c) Longitudinal color spectral Doppler US image of the SMA demonstrates turbulent flow. US image demonstrates aliasing and disturbed flow within the vessel.
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Figure 1b. Representative intraoperative (a) transverse and (b) longitudinal color Doppler US images of the SMA. Diffuse symmetric hypoechoic halo (cursors) surrounds the patent lumen. The vessel is narrowed and is 1.3-1.7 mm in diameter. (c) Longitudinal color spectral Doppler US image of the SMA demonstrates turbulent flow. US image demonstrates aliasing and disturbed flow within the vessel.
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Figure 1c. Representative intraoperative (a) transverse and (b) longitudinal color Doppler US images of the SMA. Diffuse symmetric hypoechoic halo (cursors) surrounds the patent lumen. The vessel is narrowed and is 1.3-1.7 mm in diameter. (c) Longitudinal color spectral Doppler US image of the SMA demonstrates turbulent flow. US image demonstrates aliasing and disturbed flow within the vessel.
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IMAGING FINDINGS
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An intraoperative US image of the SMA showed the vessel and demonstrated flow within the vessel lumen. The SMA was patent, but the lumen was markedly narrowed and was no more than 1.7 mm in diameter (Fig 1). The SMA was nearly occluded at the division of the SMA into its branches at US. A thick circumferential hypoechoic wall or "halo" was seen. The hypoechoic wall thickening was diffuse and symmetric. There was no displacement of the vessel because of a mass. No focal aneurysms were identified in the proximal portion of the vessel. At spectral Doppler US (Fig 1c), turbulent flow was demonstrated.
Computed tomography (CT) was performed after a bypass graft of the iliac artery to the SMA was successfully placed. On the CT scan, the proximal SMA wall was concentrically thickened (Fig 2a). There was distal focal occlusion of the SMA (Fig 2b). The bypass graft of the iliac artery to the SMA entered the SMA several centimeters distal to this occlusion.
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Figure 2a. (a,b) Selected transverse images obtained at contrast material-enhanced helical CT demonstrate concentric thickening (arrows) of the SMA seen on a, with occlusion (arrows) observed on b, a more caudal image.
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Figure 2b. (a,b) Selected transverse images obtained at contrast material-enhanced helical CT demonstrate concentric thickening (arrows) of the SMA seen on a, with occlusion (arrows) observed on b, a more caudal image.
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DISCUSSION
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This patient had bowel ischemia and a history of unilateral headaches and vision loss at presentation. Concentric hypoechoic thickening of the SMA wall was seen at intraoperative US. The differential diagnosis included vasculitis, atherosclerosis, and malignant involvement of the vessel. The US findings and the clinical history are most consistent with a type of vasculitis, most likely giant cell arteritis.
Giant cell arteritis is the most common primary systemic vasculitis and typically affects those who, such as this patient, are older than 50 years of age. There are two common constellations of findings in giant cell arteritis: temporal arteritis and polymyalgia rheumatica. Symptoms of temporal arteritis include unilateral headache, facial pain, jaw claudication, or loss of vision. Temporal arteritis is a common manifestation of giant cell arteritis and may be confirmed by using temporal artery biopsy (1). Histologic analysis may reveal granulomatous inflammatory changes. A temporal artery biopsy in this patient demonstrated giant cells and chronic inflammatory changes in the temporal artery, findings that are consistent with temporal arteritis. In many patients, the clinical manifestation and laboratory values are suggestive of a diagnosis of temporal arteritis and a biopsy is unnecessary. However, in cases of clinical uncertainty, biopsy may be required for a definitive diagnosis.
A characteristic gray-scale US finding of giant cell arteritis involvement of the temporal artery is a diffusely thickened hypoechoic arterial wall or halo. Color and spectral Doppler US may depict turbulent flow and stenosis of the affected vessel. Schmidt (2) states that the halo must be circumferential and must be demonstrated in two planes. In his large series, the sensitivity and specificity of a halo and stenosis detection by using duplex US to diagnose giant cell arteritis were 71% (n = 56) and 99% (n = 364), respectively, on the basis of a clinical diagnosis of giant cell arteritis or 80% (n = 39) and 91% (n = 47), respectively, on the basis of a histologic diagnosis. In his series, only two of 364 patients with a clinical diagnosis of polymyalgia rheumatica had the halo in a patent temporal artery and had histologic findings negative for giant cell arteritis of the temporal artery (2). There is some debate about whether the combination of gray-scale and color Doppler US findings is specific enough to replace biopsy for the diagnosis of giant cell arteritis (3).
Extracranial vessel involvement is not an uncommon finding in patients with temporal arteritis at autopsy, although clinical manifestation due to the effects of the extracranial vessel involvement is rare (4). Large-vessel involvement, especially that of the extremities, has been described, including the brachiocephalic artery and carotid artery (5). A halo, similar to that observed in our case, has been described in the axillary and brachial arteries. The hypoechoic halo in these vessels became hyperechoic within 1 year after treatment. It is hypothesized that this was due to resolution of acute edema and development of sclerosis, fibrous intimal hyperplasia, scarred media of the vessel wall, and fibrosis. Mild thickening, as well as subtotal occlusions, of the brachial and subclavian arteries has been reported (5). In an article by Schmidt et al (6), an interesting note was the resolution of the halo in a mean of 16 days after the commencement of corticosteroid therapy.
Since the SMA is an unusual location for giant cell arteritis, other vasculitides should also be considered in the differential diagnosis. The SMA may be involved in large-vessel vasculitides, such as Takayasu arteritis or giant cell arteritis. Medium-vessel vasculitides, such as polyarteritis nodosa, also affect the viscera. Small-vessel vasculitides also may cause bowel ischemia or hemorrhage. In addition, systemic lupus erythematosus, rheumatoid arthritis, and other vasculitides, such as Henoch-Schönlein purpura, Kawasaki disease, and Churg-Strauss angiitis, may affect the gastrointestinal tract (7).
The most likely type of vasculitis in this case, other than giant cell arteritis, is polyarteritis nodosa. In a case report of polyarteritis nodosa, the investigators described a halo similar to that which Schmidt (2) observed in his large series. In fact, in polyarteritis nodosa, a halo may rarely manifest in the temporal artery (8). However, polyarteritis nodosa usually affects small to medium vessels (1), and this patient did not have the typical aneurysms associated with polyarteritis nodosa.
There is little evidence to support the involvement of other vasculitides in this case. Takayasu aortitis affects the large arteries of the aorta and may cause bowel ischemia (1) but is statistically less likely to occur in elderly patients. A hyperechoic thickened vessel wall is reported in Takayasu aortitis, rather than the hypoechoic appearance as observed in this patient (9). Also, Takayasu aortitis is most common in Asia and the Far East (1). Henoch-Schönlein purpura most often affects children. Furthermore, signs of purpura were not present in this patient. Kawasaki disease is also most prevalent in children and is therefore an unlikely diagnosis (1). This patient had no evidence of the asthma or peripheral eosinophilia, which are often associated with Churg-Strauss angiitis. The absence of upper respiratory or renal involvement makes a diagnosis of Wegener granulomatosis unlikely. Diagnosis of other vasculitides, such as systemic lupus erythematosus or rheumatoid arthritis, was not supported by other signs or symptoms.
Although present in this case, gastrointestinal vasculitis is less common in giant cell arteritis than it is in other vasculitides (10); it is more commonly described in polyarteritis nodosa, systemic lupus erythematosus, and rheumatoid arthritis (11). However, several cases of bowel ischemia have been attributed to giant cell arteritis (4,7,10-13). In the uncommon event that giant cell arteritis affects the bowel, bowel infarction is most commonly described (4,7,10,12,13). Other complications include bowel perforation (11).
It is important to distinguish typical irregular atherosclerotic intimal changes with calcified plaque from the hypoechoic wall thickening seen at US with a vasculitis, such as giant cell arteritis. In older patients, atherosclerotic disease is a common cause of stenosis of the visceral vessels. However, especially in a younger patient, the possibility of vasculitis must be considered.
A malignancy infiltrating into the SMA, such as pancreatic adenocarcinoma, should also be considered. Involvement of the SMA because of a malignancy is another possible cause of stenosis of the artery with a surrounding mass. However, a malignancy is unlikely in this particular case, since imaging revealed smooth symmetric circumferential diffuse thickening without an extravascular abnormality. Careful evaluation of laboratory values and the pancreas would be warranted if there was additional evidence of malignancy.
In summary, giant cell arteritis is the most likely diagnosis on the basis of the US findings of concentric hypoechoic thickening of the SMA in this elderly patient with a history of headaches and visual defects. To our knowledge, the US appearance of giant cell arteritis in the SMA has not been previously described. However, the findings are suggestive of vasculitis even in the absence of a prior report. The US findings could represent those of one of many vasculitides or possibly those of a malignancy. Because giant cell arteritis rarely involves the bowel, a temporal artery biopsy was necessary for a final diagnosis in this patient. However, the US appearance of the SMA is consistent with abnormalities seen in other vessels in patients with giant cell arteritis.
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FOOTNOTES
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Part 1 of this case appeared 4 months previously and may contain larger images.
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REFERENCES
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- Savage CO, Harper L, Cockwell P, Adu D, Howie AJ. ABC of arterial and vascular disease: vasculitis. BMJ 2000; 320:1325-1328.[Free Full Text]
- Schmidt WA. Doppler ultrasonography in the diagnosis of giant cell arteritis. Clin Exp Rheumatol 2000; 18:S40-S42.[Medline]
- Puechal X, Chauveau M, Menkes CJ. Temporal Doppler-flow studies for suspected giant-cell arteritis (letter). Lancet 1995; 345:1437-1438. [Erratum: Lancet 1995; 346:62.].[Medline]
- Phelan MJ, Kok K, Burrow C, Thompson RN. Small bowel infarction in association with giant cell arteritis. Br J Rheumatol 1993; 32:63-65.[Abstract/Free Full Text]
- Schmidt WA, Kraft HE, Borkowski A, Gromnica-Ihle EJ. Color duplex ultrasonography in large-vessel giant cell arteritis. Scand J Rheumatol 1999; 28:374-376.[CrossRef][Medline]
- Schmidt WA, Kraft HE, Vorpahl K, Volker L, Gromnica-Ihle EJ. Color duplex ultrasonography in the diagnosis of temporal arteritis. N Engl J Med 1997; 337:1336-1342.[Abstract/Free Full Text]
- Burke AP, Sobin LH, Virmani R. Localized vasculitis of the gastrointestinal tract. Am J Surg Pathol 1995; 19:338-349.[Medline]
- Francois M, Koussa D, Declerck D. Periarterial temporal halo in the ultrasonographic image of polyarteritis nodosa. Presse Med 1999; 28:133. [French].
- Maeda H, Handa N, Matsumoto M, et al. Carotid lesions detected by B-mode ultrasonography in Takayasu’s arteritis: "macaroni sign" as an indicator of the disease. Ultrasound Med Biol 1991; 17:695-701.[CrossRef][Medline]
- Srigley JR, Gardiner GW. Giant cell arteritis with small bowel infarction: a case report and review of the literature. Am J Gastroenterol 1980; 73:157-161.[Medline]
- Smith JA, O’Sullivan M, Gough J, Williams BD. Small-intestinal perforation secondary to localized giant-cell arteritis of the mesenteric vessels. Br J Rheumatol 1988; 27:236-238.[Abstract/Free Full Text]
- Krant JD, Ross JM. Extracranial giant cell arteritis restricted to the small bowel. Arthritis Rheum 1992; 35:603-604.[Medline]
- Hassan WU, Daymond TJ. Small bowel infarction in association with giant cell arteritis (letter). Br J Rheumatol 1993; 32:942.[Free Full Text]
Congratulations to the 95 individuals who submitted the most likely diagnosis (credit given for giant cell arteritis or temporal arteritis) for Diagnosis Please, Case 58. The names and locations of the individuals, as submitted, are as follows:
- Neal D. Abdullah, MD, Bloomington, Ind
- Gholamali Afshang, MD, Tinley Park, Ill
- Dr Jorge Ahualli, Tucumán, Argentina
- Albert J. Alter, Madison, Wis
- Edward L. Baker, MD, San Francisco, Calif
- Ken Baliga, Rockford, Ill
- Richard Beedie, Auckland, New Zealand
- Debra M. Berger, MD, New York, NY
- Siva R. Bodagala, MD, Houston, Tex
- Eric L. Bressler, MD, Minnetonka, Minn
- Dr Tirso Cascajares Murillo, Los Mochis, Mexico
- Nelson M. G. Caserta, MD, São Paulo, Brazil
- Thomas S. Chang, MD, Pittsburgh, Pa
- Daniel M. Chernoff, MD, PhD, Saratoga Springs, NY
- Timothy J. Clark, MD, Greenville, NC
- James W. Cole, MD, Cincinnati, Ohio
- Federico Dalla Torre, MD, Rio Negro, Argentina
- Wagner D. de Paula, MD, Brasilia, DF, Brazil
- Manoel de Souza Rocha, MD, São Paulo, Brazil
- Jacques Demers, MD, La Pocatière, Québec, Canada
- María Jesús Díaz Candamio, La Coruña, Spain
- Shella Farooki, MD, Dublin, Ohio
- Arie Franco, Livingston, NJ
- Akira Fujikawa, Tokyo, Japan
- Martin García Pérez, Almería, Spain
- Douglas Gardner, MD, Windsor, Ontario, Canada
- Ted A. Glass, MD, Fredericksburg, Va
- Mark Goldshein, MD, Andover, Mass
- Thomas V. Greaney, Kerry, Ireland
- Ferris M. Hall, MD, Boston, Mass
- Marla R. Hersh, MD, Tampa, Fla
- Dr John A. Holemans, FRCR, Liverpool, England
- Man Hon, MD, Mineola, NY
- Alberto Iaia, MD, Wilmington, Del
- Waleed Ibrahim, MD, Detroit, Mich
- Ameer Jaff, MD, Paris, France
- Mary Ann Johnson, MD, FRCPC, Edmonton, Alberta, Canada
- Nurettin Katranci, MD, Antalya, Turkey
- Douglas S. Katz, MD, Mineola, NY
- Eric Kinder, MD, Seattle, Wash
- Mitchell A. Klein, MD, Milwaukee, Wis
- Steven A. Klein, MD, Shrewsbury, Mass
- Arlene Klink, MD, Irvine, Calif
- Kenyon Kopecky, MD, Zionsville, Ind
- Glenn Krinsky, New York, NY
- Robert L. Kushner, Jr, Atlanta, Ga
- Mario Laguna, West Allis, Wis
- Roger Lao, MD, Westbury, NY
- John T. Lim, MD, Newport Coast, Calif
- Dr Smita Mahajan, Ulhasnagar, Bombay, India
- Stephen Manghisi, MD, Closter, NJ
- N. B. S. Mani, MD, Nassau, Bahamas
- Frank McKowne, MD, Vancouver, Wash
- Flavia Mendez, MD, Porto Alegre, Brazil
- Manabu Minami, MD, Tokyo, Japan
- Eduardo Mondello, MD, Buenos Aires, Argentina
- Carlos J. Nassar, MD, Humacao, Puerto Rico
- Tammam Nehme, Milwaukee, Wis
- Mike O’Loughlin, MD, West Hartford, Conn
- Sanford M. Ornstein, MD, Phoenix, Ariz
- Ann Owen, MD, Murfreesboro, Tenn
- Harish Panicker, MD, Detroit, Mich
- Narendrakumar P. Patel, MD, Newburgh, NY
- Lisa K. Quane, Orange, Calif
- Shawn P. Quillin, MD, Charlotte, NC
- James Ravenel, MD, Charleston, SC
- Enrique Remartinez Escobar, Melilla, Spain
- Luiz Antonio Rossi, MD, São Paulo, Brazil
- Dr N. Saravanan, Chandigarh, India
- Dr Mustafa Secil, Izmir, Turkey
- Matt Shapiro, MD, Lowell, Mass
- Taro Shimono, MD, Osaka, Japan
- Michael K. Silberman, Durham, NC
- Paolo Siotto, MD, Cagliari, Italy
- Darrin S. Smith, MD, Fresno, Calif
- David F. Sobel, MD, La Jolla, Calif
- Mladen Sostaric, Brezice, Slovenia
- Simon Strauss, MBChB, Kfar Shmaryahu, Israel
- Kouichi Sugiyama, Hamamatsu, Japan
- Douglas L. Teich, MD, Brookline, Mass
- D. Dean Thornton, MD, Birmingham, Ala
- Stephen Y. C. To, MD, Pacific Palisades, Calif
- Eugene Tong, MD, Austin, Tex
- Meriç Tüzün, Ankara, Turkey
- Piet K. Vanhoenacker, MD, Aalst, Belgium
- Raimo Virkki, Turku, Finland
- Christopher Vittore, MD, Rockford, Ill
- Zhen Jane Wang, MD, San Francisco, Calif
- Thomas Waslen, Saskatoon, Saskatchewan, Canada
- Jeff West, MD, Jacksonville, Fla
- David J. Wright, MD, Lake Oswego, Ore
- Benjamin M. Yeh, MD, San Francisco, Calif
- Stanko Yovichevich, MD, Sydney, Australia
- Joe Yut, Olathe, Kan
- Jeffrey H. Zapolsky, MD, Oshkosh, Wis
TOP
HISTORY
IMAGING FINDINGS
