Rheumatology Advance Access originally published online on January 23, 2007
Rheumatology 2007 46(3):554-555; doi:10.1093/rheumatology/kel392
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Acute coronary syndrome in Takayasu arteritis without elevation of acute phase parameters
Department of Rheumatology, University Hospital of Schleswig-Holstein, Campus Lübeck and Department for Internal Medicine, Rheumatology and Immunology, Rheumaklinik Bad Bramstedt, 1Institute of Pathology, University Hospital of Schleswig-Holstein, Campus Lübeck, 2Medical Clinic I, Clinic of the City of Wolfsburg, Teaching Hospital of the University of Göttingen and 3Clinic for Radiological Therapy and Nuclear Medicine, University Hospital of Schleswig-Holstein, Campus Lübeck, Germany.
Correspondence to: K. Ahmadi-Simab, University Hospital of Schleswig-Holstein, Campus Lübeck and Rheumaklinik Bad Bramstedt, Oskar-Alexander-Str. 26, D-24576 Bad Bramstedt, Germany. E-mail: ahmadi{at}rheuma-zentrum.de
SIR, Takayasu arteritis (TA) is a primary systemic vasculitis of the aorta and its major branches. Cardiac involvement is seen in around 22% of cases and occurs usually later during the disease course. We report on a patient with coronary arteritis as the first manifestation of TA.
A female 36-yr-old patient was taken into hospital because of a primary onset of retrosternal pressure in resting position. Coronary angiography showed a long-range subtotal excentric 80% stenosis of the anterior descending branch of the left coronary artery (LAD) and the patient was referred to coronary artery bypass surgery. Erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) were normal. After 8 months, a repeat coronary angiogram revealed a new stenosis of the circumflex branch of the left coronary artery.
After 1 week later, the patient was transferred to our department with a history of arthralgias and myalgias for about 6 months. In view of the coronary artery disease and the lack of cardiovascular risk factors, a diagnosis of a large vessel vasculitis was taken into consideration, but serological markers of inflammation were again normal. A 18-fluorodeoxyglucose positron emission tomography ([18F]FDG-PET) showed a hypermetabolic activity of the aorta arch (Fig. 1), femoral arteries and popliteal arteries. Biopsy samples of the coronary artery segment obtained during bypass surgery were re-examined and were suggestive of a diagnosis of TA.
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Other types of primary or secondary large vessel vasculitis were considered to be unlikely: polyarteritis nodosa involves medium-sized vessels, but without a giant cell reaction; Kawasaki's syndrome typically affects coronary arteries, however, it is an acute febrile disease of childhood age very rarely producing fibrinoid necrosis as in our case but rather multiple aneurysms [1]. Giant cell arteritis (GCA) may result in an identical histological picture, but affects patients above the age of 50 [2]. Vessel involvement in Cogan's syndrome may be identical to TA, but the characteristic ocular and vestibular symptoms were lacking in our case. There were no clinical or serological signs suggestive of secondary forms of vasculitis.
In summary, the angiographically documented stenoses of the coronary vessels were compatible with a coronary arteritis due to TA. In view of the severe complication in her past history and the presently active vasculitis, the patient was treated with intravenous cyclophosphamide pulse therapy (15 mg/kg/day in 21-day intervals + prednisolone, initially 1 mg/kg/day). After a total of six cyclophosphamide pulses a repeat PET-examination showed no enhancement in the previously affected vessels suggesting a remission of TA. Therapy was then switched to azathioprine for maintenance of remission. During a follow-up time of 6 months, relapses were not observed and the patient did not complain of angina pectoris. A repeat coronary angiogram obtained 1 month after treatment with cyclophosphamide did not show new stenoses.
An elevation of serological acute phase parameters such as ESR and CRP is generally considered as an important criterion for the diagnosis of TA. However, in one study involving 32 patients with TA an increased ESR was seen in only 78% of all cases [3]. Histopathological studies also demonstrated that in 
40% of patients with TA who go into a clinical remission with normal acute phase proteins, an active vasculitis can be seen in surgically obtained vessel specimens [4, 5]. In the case reported here, no increase of ESR and CRP was seen during active disease and a diagnosis of TA was suggested by coronary angiography, and [18F]FDG-PET only. However, the changes visible in the coronary angiography did not deliver distinct hints for the differential diagnosis of a vasculitis. Retrospectively, the diagnosis in this case was ascertained by histological examination of an affected coronary artery.
The case in hand illustrates the value of non-invasive imaging methods in diagnosing large vessel vasculitides involving big vessels. In a study with 18 patients with TA the [18F]FDG-PET-examination showed a sensitivity of 92% and a specificity of 100% [6]. The [18F]FDG-PET offers an appropriate method for early diagnosis and appears also helpful in therapy monitoring of the TA, especially if serological inflammation parameters cannot be taken into account [7, 8]. In comparison to cardiac MR and CT, [18F]FDG-PET does not allow an evaluation of the coronary vessels, but it is useful for assessment of the whole aorta and is primary branches with a reasonable effort of time.
In conclusion, a diagnosis of TA should be considered in young patients with coronary vessel disease in which classical risk factors of atherosclerosis are lacking. Our experience reported here suggests that [18F]FDG-PET can be helpful to establish the diagnosis, but further studies are required to address this hypothesis.
The authors have declared no conflicts of interest.
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[Abstract/Free Full Text]
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