Thoracoabdominal aortic aneurysm associated with systemic sclerosis

Sir, Aortic aneurysm in association with systemic sclerosis (SSc) has never been reported so far. We describe for the first time an SSc patient who developed a huge thoracoabdominal aortic aneurysm and improved after surgical treatment. A 34-yr-old woman was admitted to our hospital in April 2002 for investigation of pleuritic chest pain. She underwent aortic valve replacement and aneurysmectomy due to aortic insufficiency associated with an aortic root aneurysm in January 1994. She presented with Raynaud's phenomenon and digital ulcers, and was referred to the rheumatology unit. Since 1992 she had suffered from Raynaud's phenomenon, polyarthralgia, and skin sclerosis of the hands, forearms and face. During the following years she presented numerous episodes of ischaemic lesions on the fingertips. She had no bruit and her blood pressure was at 126/78 and 122/74 mmHg in the right and left arms, respectively. There was no history of drug allergy, alcohol consumption or smoking. She presented no clinical features of Marfan's syndrome on physical examination. She showed a typical late scleroderma pattern on nailfold capillaroscopic examination, and the presence of anti-Scl70 antibody in serum. However, tests for anti-double-stranded DNA antibody, anti-Ro/La antibodies, anti-Sm/RNP antibodies, anti-cardiolipin antibodies, lupus anticoagulant, ANCA and serum complements and the Venereal Disease Research Laboratory (VDRL) test were all negative. Hand radiographs revealed acro-osteolysis of both fingers, but chest radiographs and pulmonary function test were normal. Her blood cholesterol, high-density lipoprotein, triglyceride, glucose and renal function were all normal. She was classified as having SSc, limited scleroderma, according to the 1980 American Rheumatology Association (ARA) criteria for SSc [1]. Computed tomography (CT) and an aortogram (Fig. 1) confirmed a thoracoabdominal aortic aneurysm, which started immediately proximal to the left subclavian artery and extended to 3 cm proximal of the aortic bifurcation. Surgery was performed and pathological specimens were obtained. These demonstrated a mild to moderate plasma cell infiltrate consistent with severe atherosclerosis, without evidence of dissection (Fig. 2). The conclusion of the pathologist was that the fundamental alteration was atherosclerosis, not arteritis or hereditary collagen disorder. Two years later, the patient was clinically stable and CT showed normalization of the thoracoabdominal aorta. Aortogram showing a fusiform thoracoabdominal aortic aneurysm extending from proximal part of left subclavian artery to proximal 3 cm of aortic bifurcation. Atheromatous plaque showing fibrous cap and a central necrotic core, containing cholesterol clefts, scattered inflammatory cells, and calcification. Haematoxylin and eosin, ×40. Prominent vascular abnormalities in SSc are noted in the microvascular systems, such as capillaries and small arterioles [2]. Macrovascular disease is also more common in SSc compared with unaffected subjects [3, 4], but is usually limited to the ulnar or cerebral arteries [5, 6]. There are similarities in clinical symptoms between SSc and atherosclerotic disease with digital and lower limb ulcers [3]. In addition, the vascular pathological modifications and pathogenesis of SSc are strikingly similar to those of atherosclerosis [7–9]. A previous study suggested that there is a progressive and highly significant reduction in the elastic properties of the carotid artery of SSc patients compared with non-SSc subjects [10]. Also, it is well known that arterial elastic properties are good markers of early atherosclerosis [11]. Therefore, it seems likely that altered vessel stiffness may be associated with macrovascular arterial complications in SSc patients. It remains unanswered at present whether these macrovascular diseases of SSc are atherosclerotic in nature. Nevertheless, the patient in our report may represent an example of SSc developed macrovascular complicated by severe atherosclerotic changes. The authors have declared no conflicts of interest.