British Journal of Radiology (2004) 77, 607-609
© 2004 British Institute of Radiology
doi: 10.1259/bjr/22460193
Use of colour duplex ultrasound to diagnose giant cell arteritis in a case of visual loss of uncertain aetiology
D J A Butteriss, BSc, MBBS (Hons)1,
L Clarke, MBBS2,
M Dayan, MBBS, FRCS2 and
D Birchall, MBChB, FRCR1
1 Department of Neuroradiology, Regional Neurosciences Centre, Newcastle General Hospital, Westgate Road, Newcastle upon Tyne NE4 6BE and 2 Department of Ophthalmology, Royal Victoria Infirmary, Newcastle upon Tyne, UK
Correspondence: Dr D Birchall
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Abstract
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Giant cell arteritis is a common condition that can result in permanent visual loss. It has traditionally been diagnosed by invasive temporal artery biopsy in cases of clinical suspicion. The findings of colour duplex ultrasound have recently been described. We report the use of duplex ultrasound to diagnose temporal arteritis, with clinicopathological correlation, and discuss the possible application of this non-invasive technique to the management of giant cell arteritis.
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Introduction
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Giant cell arteritis (GCA) is a relatively common condition that can result in irreversible visual loss if untreated. Final confirmation or exclusion of the diagnosis usually requires temporal artery biopsy. Recently there have been studies of the use of duplex ultrasound to diagnose temporal arteritis. In the following case we describe the use of duplex ultrasound in the diagnosis of a case of GCA, with histopathological confirmation of the diagnosis, and discuss the possible applications of the technique.
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Case report
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An 81-year-old woman was admitted under the care of the ophthalmologists complaining of a 1 day history of decrease in vision in her right eye. There was no associated pain; headache, myalgia or scalp tenderness and she denied jaw claudication. Direct questioning revealed a vague history of "viral illness" 5 weeks prior. On examination visual acuity was hand movements in the right, 6/9 left. She was found to have a relative afferent papillary defect on the right side and a swollen, pale optic disc on fundoscopy. Her superficial temporal arteries were felt to be nodular but non-tender bilaterally, with decreased pulsation on the right side. Blood tests revealed an erythrocyte sedimentation rate (ESR) of 34 mm h–1 (within the normal range in this age group) and an elevated C-reactive protein titre of 36 mg l–1 (normal range 0–5 mg l–1).
A diagnosis of right anterior ischaemic optic neuropathy, presumed secondary to GCA was made, and high dose intravenous steroid therapy instituted. Duplex ultrasound of the right superficial temporal artery was performed and showed wall thickening with an echolucent "halo" and an attenuated lumen with irregular stenotic segments (Figures 1a,b
). For comparison, Figures 1c,d show the appearances in a normal, unaffected temporal artery. Appearances were felt to be highly suggestive of GCA and an abnormal segment was marked for biopsy, which was carried out under local anaesthetic the same day. Histology showed a chronic inflammatory cell infiltrate involving the full thickness of the artery wall, with multinucleate giant cells and fibromyxoid intimal thickening with associated luminal narrowing, consistent with active GCA (Figure 2).
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Figure 1. (a) Longitudinal duplex ultrasound image through the right superficial temporal artery showing irregular lumenal stenosis and mural thickening. (b) Transverse duplex ultrasound image showing the "halo" effect due to mural oedema. (c) Longitudinal duplex ultrasound image through the right superficial temporal artery in a normal control subject. (d) Transverse duplex ultrasound image through the same unaffected vessel, showing normal appearance of the arterial wall.
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Figure 2. Histological section through the right temporal artery showing inflammatory infiltrate with multinucleate giant cells. (a) Low power, (b) high power detail.
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After 6 days treatment as an inpatient, the optic disc swelling had begun to resolve. Her ESR fell to 8 mm h–1 and C-reactive protein <5 mg l–1. However there was no change in her visual acuity in either eye and she was discharged. Continuing slow reduction of oral steroid dosage, at 2 months follow-up haematological and visual parameters remained unchanged.
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Discussion
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GCA is a relatively common condition with a prevalence of approximately 1 in 500 of the over 50 population, with the incidence increasing with age. It is a medical emergency due to the possibility of irreversible visual loss. If untreated 5–20% of patients develop arteritic anterior optic neuropathy due to occlusion of the posterior ciliary arteries. This most commonly occurs in the earliest stages of the disease, and is almost unknown more than 9 months after the onset of symptoms. For this reason diagnosis and treatment of the condition must not be delayed.
The American College of Rheumatology published criteria for the classification of temporal arteritis in 1990 [1] to help discriminate between different types of vasculitis. It requires three of the following five criteria to be met: age 
ge;50 years, new onset localized headache, temporal artery tenderness or decreased pulse, ESR ge;50 mm h–1, abnormal histology. Unfortunately diagnosis is not always straightforward. As in the case we describe, symptoms may be non-specific or even largely absent and ESR may be normal. A temporal artery biopsy is not infallible, as there can be false negatives due to the presence of skip lesions. Even though it is a relatively minor procedure usually carried out under local anaesthetic it may be refused by the patient or have complications such as local infection or damage to auriculotemporal nerve branches [2]. High dose steroid therapy carries the risk of significant side effects such as electrolyte disturbance, hypertension and psychiatric disturbance acutely; diabetes, osteoporosis and adrenal suppression with long term usage. Treatment cannot therefore be instituted without adequate justification.
Recent reports have suggested that ultrasound may be a useful adjunct in the diagnosis of GCA. Puechal et al [3] described the use of Doppler ultrasound examination of the temporal, ophthalmic and facial arteries in the diagnosis of GCA and found it to have a specificity of 80% and a sensitivity of 77%, using temporal artery biopsy as the gold standard. Schmidt et al described the use of duplex ultrasound to examine the morphology and flow characteristics in temporal arteries [4, 5]. They found no significant changes in blood velocity in affected arteries, but described irregularity, stenoses, wall thickening and the presence of an echolucent halo around affected arteries due to mural oedema [6], which the authors felt was the most specific sign of arteritis when circumferential and imaged in two planes [7]. They found that duplex ultrasound abnormalities had a 93% specificity and sensitivity for diagnosing GCA. In a similar study Stammler et al [8] found that the presence of a halo was 100% sensitive and 80% specific for temporal arteritis, using temporal artery biopsy as the gold standard. More recently however, Venz et al [9] have demonstrated an indistinguishable halo in the temporal arteries of patients without GCA, showing that it is not completely specific.
In conclusion, duplex ultrasound is a recently described but little used non-invasive technique for the investigation of the temporal arteries in cases of possible GCA. In our case report a questionable case of GCA was confirmed on ultrasound and histological examination. We feel that duplex ultrasound is a rapid, simple and non-invasive method that has several applications. For example in equivocal cases or when patients refuse temporal artery biopsy a normal ultrasound may obviate the need for biopsy or unnecessary treatment, whereas an abnormal result, particularly one demonstrating both an echolucent halo and areas of stenosis, would be highly suggestive of the diagnosis. It can also be used to identify abnormal segments before biopsy to reduce the risk of a false negative result. Further study is necessary to determine if duplex ultrasound alone is sensitive and specific enough to diagnose GCA without recourse to temporal artery biopsy.
Received for publication October 16, 2002.
Revision received August 13, 2003.
Accepted for publication October 9, 2003.
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References
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- Hunder GG, Bloch DA, Michel BA, et al. The American College of Rheumatology 1990 criteria for the classification of giant cell arteritis. Arthritis Rheum 1990;33:1122–8.[Medline]
- Scott KR, Tse DT, Kronish JW. Temporal artery biopsy technique: a clinico-anatomical approach. Ophthalmic Surg 1991;22:519–25.[Medline]
- Puechel X, Chauveau M, Hilliquin P, Perrot S, Job-Deslandre C, Menkes CJ. Superficial temporal Doppler flow studies in suspected giant cell arteritis: validation of a diagnostic score. Arthritis Rheum 1994;37(Suppl. 9):S409 [abstract].
- Schmidt WA, Kraft HE, Vorpahl K, Volker L, Gromnica-Ihle E. Colour duplex ultrasonography in the diagnosis of temporal arteritis. N Engl J Med 1997;337:1336–42.[Abstract/Free Full Text]
- Kraft HE, Moller DE, Volker L, Schmidt WA. Colour Doppler ultrasound of the temporal arteries—a new method for diagnosing temporal arteritis. [German] Klin Monatsbl Augenheilkd 1996;208:93–5.
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- Schmidt WA. Doppler ultrasonography in the diagnosis of giant cell arteritis. Clin Exp Rheumatol 2000;18(Suppl. 20):S40–2.
- Stammler F, Ysermann M, Mohr W, Kuhn C, Goethe S. Value of color-coded duplex ultrasound in patients with polymyalgia rheumatica without signs of temporal arteritis. [German] Dtsh Med Wochenschr 2000;125:1250–6.[CrossRef]
- Venz S, Hosten N, Norwald K, Lemke AJ, Schroder R, Bock JC, et al. Use of high resolution colour Doppler sonography in diagnosis of temporal arteritis. [German] Rofo Fortschr Geb Rontenstr Neuen Bildgeb Verfahr 1998;169:605–8.
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Abstract
Introduction
