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We report the case of a 30 year old woman with atypical presentation of Takayasu’s disease in whom the diagnosis was first made by an abdominal computed tomography (CT) scan.
A 30 year old woman was admitted for investigation of an eight month history of lower abdominal pain. The pain was cramping in character, preceded bowel motions, and radiated to the back. She also had a four year history of general malaise with fleeting arthralgias and myalgias, and persistently raised inflammatory markers had been noted. Past medical history included alopecia, occasional mouth ulcers, and pulmonary embolism two years previously. Both her father and paternal grandfather had died of “ruptured aortic aneurysms”. Previous investigation of her abdominal pain at another hospital with an ultrasound scan had shown a small scarred right kidney.
On examination she was apyrexial, with a regular radial pulse of 84 beats/min and blood pressure of 120/70 mm Hg in both arms. There was a soft ejection systolic murmur which radiated to the neck. All peripheral pulses were palpable. Abdominal examination showed mild right iliac fossa tenderness with no guarding. Examination of the respiratory and nervous systems (including fundoscopy) were unremarkable. Investigations showed a raised erythrocyte sedimentation rate (ESR) of 88 mm/1st h and raised C reactive protein (CRP) of 77 mg/l. Perinuclear antineutrophil cytoplasmic antibodies were demonstrated by immunofluorescence, but enzyme linked immunosorbent assay (ELISA) measurements of antibodies to myeloperoxidase and proteinase 3 were normal. Serum angiotensin converting enzyme and complement levels were normal; rheumatoid factor and antinuclear antibodies were negative. An echocardiogram showed mild aortic regurgitation and a slightly dilated ascending aorta, but normal cardiac function.
To investigate the abdominal pain further abdominal CT scanning was performed. This showed a normal gastrointestinal tract, but was remarkable for diffuse thickening of the abdominal aortic wall and narrowing of the aortic lumen (fig 1). Image A also shows some evidence of calcification of the intima. Administration of intravenous contrast showed that the aortic wall was ∼1 cm thick. Image B shows encasement of the origin of the coeliac axis. As well as aortitis, this thickening might possibly represent periaortitis.
On re-examination of the patient bilateral renal bruits were noted. In view of the history of prominent inflammatory symptoms—malaise, arthralgias, myalgias; symptoms suggestive of vessel involvement—abdominal aortic narrowing causing ischaemic pain; and the physical examination, a diagnosis of Takayasu’s arteritis was considered. This was confirmed by aortography which, as well as showing the dilated ascending aorta and the narrowed abdominal aorta, also demonstrated stenoses of both subclavian and proximal renal arteries. Small aneurysms of the third and fourth order branches of the right renal artery were seen.
She was treated with pulsed intravenous methylprednisolone and discharged on oral prednisolone. After two weeks the patient reported that her pain had resolved completely and she was considerably less fatigued. At this time the ESR was 6 mm/1st h and CRP 2 mg/l.
Although a thorough history and examination can allow the diagnosis of Takayasu’s arteritis to be made, confirmation and assessment of the extent of the disease depends on arteriography. Classically, the aortogram shows both dilatation and stenosis of large arteries, but this is not always the case. However, unusually in this patient the diagnosis was suggested by CT scanning demonstrating aortitis. Recent published reports have shown that CT can be very useful in detecting Takayasu’s arteritis because it shows such details as wall thickening and perivascular oedema, which cannot be detected by arteriography.1–3 CT is also useful as a non-invasive means of monitoring patients with Takayasu’s arteritis who are undergoing treatment, to examine disease resolution or progression.
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