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