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Multiple aortic aneurysms complicated by a rupture in the systemic lupus erythematosus: A case report

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Abstract

We report the case of a 61-year-old female who suffered from systemic lupus erythematosus (SLE) and died of a ruptured abdominal aortic aneurysm (AA). She was diagnosed to have SLE at 39 years of age, and was administrated steroids and prostaglandin E2. From 52 years of age, AA, peripheral arterial occlusion, and multiple organ infarctions appeared repeatedly. At 59 years of age, she was found to be affected by antiphospholipid antibody syndrome (APS). In the following year, expansion of an abdominal AA was identified, but she was given only conservative treatment. In the next year, sudden epigastralgia and dyspnea occurred, and she died. An autopsy revealed multiple AAs up to 11 cm in diameter, one of which showed ruptures, forming a retroperitoneal hematoma. Marked atherosclerosis of the aorta was noted, and she also had aortic dissection accompanied by cystic medial necrosis (CMN). An old myocardial infarction and brain infarction were also confirmed. Although SLE with APS is common, a complication of the disease by CMN, multiple AAs, or ruptured AA has been described in several cases to date. Regarding the etiology of this complicated presentation, we presume synergistic involvement of various factors, such as atherosclerosis and CMN associated with SLE, thrombosis due to APS, and prolonged steroid therapy.

Introduction

Systemic lupus erythematosus (SLE) is an autoimmune disease with multi-organ manifestations induced by the production of autoantibodies. Although the etiology of SLE is not fully elucidated, genetic factors in combination with acquired factors such as environmental, viral, and hormonal influences have been proposed as causative agents. The peak of disease onset is between the late teens and 40s, and the ratio of females to males is 9:1. It is more common in the Asian and African population than in Caucasians [8], [13]. The long-term prognosis has greatly improved recently owing to the use of steroids and immunosuppressive agents [3], [9], [26]. However, SLE has many complications, including lupus nephritis and central nervous system (CNS) lupus. Antiphospholipid antibody is detected in 60–70% of SLE patients, and it is known that thrombosis occurs in approximately 50% of patients with high levels of this antibody [5], [12], [25]. On the other hand, the complication of vasculitis in SLE is comparatively rare except for cutaneous disorders [22]. One report documents vasculitis in 76 cases (11%) of 670 SLE patients [22]. Furthermore, aneurysm formation in SLE patients is very unusual, and only 40 cases were reported over the recent years [15]. In addition, SLE accompanied by aortic dissection and rupture is extremely rare. To date, only four cases have been reported in the literature, and all of them demonstrated cardiac tamponade [11], [18], [21], [23], [27]. We encountered a SLE patient who never demonstrated hypertension, but this case was complicated with antiphospholipid antibody syndrome (APS), multiple aortic aneurysms (AAs), and aortic dissection. The patient died of rupture of an abdominal AA.

Section snippets

Case report

We report the case of a 61-year-old woman with SLE associated with multiple aneurysms. At 30 years of age, she had arthralgia and myalgia affecting the whole body. At 39 years of age, Raynaud's symptom, arthralgia throughout the body, antinuclear antibody positivity, rash (discoid rash), and leukopenia were noticed. The patient fulfilled the criteria for a diagnosis of SLE and was successfully treated with steroids, prostaglandin E2, and antiplatelet drugs. At 52 years of age, she had a

Major findings in autopsy

The cadaver (47.3 kg, 154 cm) showed a few purpura on the skin. At autopsy, the initial laparotomy revealed a retroperitoneal hematoma, which expanded around the abdominal AA. The autopsy demonstrated multiple aneurysms: (1) a distal aortic arch aneurysm, 4 cm in diameter, showing severe atherosclerosis with calcification and the presence of cholesterol crystals; (2) a thoracic AA, 7 cm in diameter, demonstrating a variable degree of atherosclerosis and focal medial dissection (Fig. 2A) along with

Discussion

The patient was diagnosed to have SLE complicated by APS, AAs, and aortic dissection with CMN. She died of rupture of the abdominal AA.

Vascular damage associated with SLE is relatively rare. Its main histopathological feature is usually leukocytoclastic or necrotizing vasculitis, the core finding being fibrinoid degeneration [4], [22]. However, AA formation in SLE is much more uncommon, and, in general, it is not accompanied by any obvious vasculitis [15], [18], [27]. Indeed, in our case, we

Acknowledgment

This article was supported in part by grants (2005–2008) from the Ministry of Health, Labour, and Welfare of Japan

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