A case of nondissecting abdominal aortic aneurysm associated with systemic lupus erythematosus

Anoushka Rapondjieva, Nedialka Dobreva and Boriana Delijska

First Nephrology Clinic, Medical University, Sofia, Bulgaria

Sir,

The most frequent cardiovascular manifestations of systemic lupus erythematosus (SLE), namely hypertension and pericardial, myocardial and endocardial changes, are well known. Recently, with prolonged patient survival and improved non-invasive imaging techniques, new vascular complications of the disease have been observed. Thus several cases of dissecting aortic aneurysm associated with SLE have been reported [13]. We describe here a case of nondissecting abdominal aortic aneurysm in a young woman with a 5-year history of SLE.

Case.

In November 1996, a 25-year-old woman was hospitalized in our clinic for evaluation of severe nephrotic syndrome, hypertension, and renal failure persisting for about 2 years. SLE was diagnosed, based on the presence of arthritis, anaemia (relatively severe for the degree of renal failure), diffuse proliferative lupus nephritis (type IV of WHO classification), and positive ANA and anti-ds-DNA antibodies. A combined treatment with corticosteroids for 9 months (cumulative dose 16 g), cyclophosphamide for 3 months, heparin for a month, and two sessions of plasmapheresis were given and clinical and serological remission was achieved. The blood pressure was controlled by a combination of ACE inhibitor, calcium channel blocker and beta blocker. One year later corticosteroid therapy was resumed over a period of 4 months because of relapse of anaemia and impaired renal function.

In August 1999, an oval, pulsing, painless periumbilical mass was discovered during routine examination in our clinic. Abdominal sonography showed an infrarenal abdominal aortic aneurysm, extending to the proximal parts of the common iliac arteries, of a size of 4.7–5.5 cm. The aortic wall was diffusely enlarged and a massive thrombus was seen on its back part (Figure 1). We failed to find positive ANCA but anti-ds-DNA and anti-ss-DNA antibodies were present (1+) and circulating immune complexes were elevated to 4.1 U (normal value <3 U). Serum cholesterol was 5.67 mmol/l. The remaining clinical and laboratory tests were normal. Blood pressure was well controlled by Captopril 25 mg/day and the patient's progress was monitored in the outpatient clinic. In February 2000, an asymptomatic enlargement of the aneurysm size to 6 cm was found on repeat ultrasound examination. A surgical treatment was proposed but the patient refused. Therefore, prednisolone 60 mg/day was given for 20 days, followed by a gradual reduction of the dose. Three months later the immunological tests became negative whereas a new abdominal sonography showed no change of the aortic aneurysm. The patient had no complaints and was continued on antihypertensive and antiplatelet treatment.

Comment.

The improved surveillance as well as a more pathogenetically orientated treatment from the early stages of SLE improved its prognosis and prolonged the survival of patients with this disease. Recently, increased attention has been paid to the cardiovascular complications of SLE. Since 1969, when Bernhard reported the first case of an aortic aneurysm in a patient with SLE, we could find only five reported cases of nondissecting abdominal aortic aneurysms associated with SLE [4,5]. Since the patients were asymptomatic, these aneurysms were discovered occasionally as an unexpected finding of abdominal ultrasonography or CT scan or during the physical examination. In our case the diagnosis was confirmed by abdominal sonography and other imaging techniques were not used because the patient refused surgical treatment.

The mechanisms of aortic aneurysm formation are not clear. Several factors such as hypertension, accelerated atherosclerosis or inflammation of the aortic wall due to autoimmune vasculitis of vasa vasorum, and prolonged corticosteroid therapy, are discussed. Elevated blood pressure is probably an important factor because all cases reported with an association of SLE and aortic aneurysm had hypertension. The most frequent histological finding was media necrosis. It was generally attributed to long-term steroid administration. Atherosclerotic changes of the aortic wall have been found in some cases [2,5] while perivascular infiltration as a major morphological finding have been described in others [3]. Our patient had severe hypertension at the manifestation of the disease and this could have played a significant role in aneurysm formation. The short duration of SLE and steroid therapy, the lack of hypercholesterolaemia, the enlarged aortic wall on sonography, and the positive immunological tests make us hypothesize that a vasculitic process played a central role in aneurysm formation in our case. Unfortunately this hypothesis could not be confirmed by morphological examination.

It is generally accepted that the risk of aortic rupture is substantial for aneurysms of 5 cm or more in diameter. We consider that the association with SLE is an additional risk factor for the dissection and rupture of an existing aortic aneurysm. In fact, the diameters of dissected aortic aneurysms reported in patients with SLE were between 3 and 6 cm. Therefore, we propose that a surgical correction should be performed earlier in SLE patients than in patients without systemic disease. Several cases of successful surgery have been reported in such situations [3,5].

In conclusion, the awareness of an aortic aneurysm as a possible complication of SLE and the use of noninvasive imaging methods may allow the prevention of a life-threatening complication in such patients.

References

  1. Nosaka S, Yamauchi M, Sasaki T, Ku K, Hanada T, Tamura K. Abdominal aortic aneurysm rupture in systemic lupus erythematosus. J Cardiovasc Surg1999; 40: 59–61[ISI][Medline]
  2. Choi KH, Rim S-J, Lee SK, Jang BC, Cho SH. Dissecting aortic aneurysm with aortic-valve insufficiency in systemic lupus erythematosus. Nephrol Dial Transplant1999; 14: 969–973[Free Full Text]
  3. Hussain KMA, Chandna H, Santhanam V, Sehgal S, Jain A, Denes P. Aortic dissection in a young corticosteroid-treated patient with systemic lupus erythematosus. Angiology1998; 49: 649–652[ISI][Medline]
  4. Khan AS, Spiera H. Association of aortic aneurysm in patients with systemic lupus erythematosus: a series of case reports and a review of the literature. J Rheumatol1998; 25: 2019–2021[ISI][Medline]
  5. Marubayashi S, Sugi K, Ishiyama K, Fukuma K, Okada K, Hinoi T et al. A case of abdominal aortic aneurysm associated with systemic lupus erythematosus. Hiroshima J Med Sci1998; 47: 85–87[Medline]




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