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Rheumatology 2001; 40: 472-473
© 2001 British Society for Rheumatology
Letters to the Editor |
Abdominal aortic aneurysm with lumbar vertebral erosion in Behçet's disease revealed by low back pain: a case report and review of the literature
Internal Medicine Department, Military Hospital Mohamed V, Rabat, Morocco
SIR, Erosion of the lumbar vertebrae due to an abdominal aortic aneurysm is rare. It has been reported in only three cases, in two of which it was related to Behçet's disease (BD) [1–3].
We encountered recently the case of a 39-yr-old man who was admitted to our department for low back pain that had persisted for 4 months. He was known to have had BD since 1996. The diagnosis was based upon recurrent episodes of oral and genital aphthosis, pseudofolliculitis, a positive pathergy test and two episodes of left knee arthritis. He was treated with colchicine 1 mg/day. This low back pain, which appeared suddenly, had an inflammatory character and did not respond to analgesics or non-steroidal anti-inflammatory drugs. It was associated with a weight loss of more than 8 kg.
Physical examination was normal except for a periumbilical pain caused by deep palpation. No oral or genital ulceration was found.
The erythrocyte sedimentation rate was 112 mm in the 1st hour, and there were 14 000 white blood cells/mm3 (80% neutrophils). Blood cultures and the tuberculin skin test were negative.
Lumbar spine X-rays were normal. Abdominal ultrasound and Doppler exploration showed a voluminous aneurysm of the abdominal aorta. Aortography coupled to abdominal computed tomography scanning confirmed an infrarenal aortic aneurysm measuring 40x60 mm located 10 mm from the aortic bifurcation and eroding the second lumbar vertebra (Fig. 1
). Renal, digestive and coronary arteriographies were normal.
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The patient was referred to the vascular surgical department, where vascular reconstruction of the abdominal aorta was performed. A prosthesis was sutured end-to-end to the infrarenal aorta and the origin of the primitive iliac arteries. Pathological examination of the resected aortic wall showed a non-specific inflammatory infiltrate compatible with BD vasculitis. Perivascular infiltrates of lymphocytes and plasma cells were present. The patient was treated with coumarin 4 mg/day, azathioprine 50 mg t.i.d. and prednisone 50 mg/day, the dose of which was reduced progressively. The outcome was good: 10 months after the intervention the patient was very well and completely asymptomatic.
BD is a multisystem disorder that is commonly seen in Japan and the Mediterranean region. The syndrome is characterized by the triad described first by Behçet in 1937: oral ulceration, genital ulceration and uveitis. Additional features, now well recognized, are synovitis, cutaneous vasculitis resembling erythema nodosum, meningoencephalitis and vascular involvement. The diagnosis remains clinical, as there are no pathognomonic serological or pathological tests. It is based upon five major criteria: recurrent oral ulcerations in combination with genital ulceration, eye lesions, skin lesions or a positive pathergy test [4]. The appearance of new clinical features months or years after onset is common. The most important complications are uveitis, meningoencephalitis and large-vessel disease.
Vascular involvement has been reported in 7.7–60% of cases; 25% of such lesions affect the venous system exclusively, whereas only 7% affect the arterial system. The majority of patients with vascular involvement (68%) present with both venous and arterial lesions [5, 6]. The vascular lesions in BD include arterial and venous occlusions and the formation of aneurysms and pseudoaneurysms, or a combination of these conditions. The most frequently reported vascular complication is thrombosis of the large veins of the limbs and superior or inferior caval vein syndrome.
Arterial involvement is less common and consists mostly of aneurysm and pseudoaneurysm formation or arterial occlusion. Occlusion and aneurysms of the large systemic arteries can cause life-threatening or fatal illness from limb ischaemia, stroke, renal hypertension or aortic aneurysm. Aneurysms of the large arteries, such as the abdominal aorta and the pulmonary artery, are reported most frequently, although nearly every major artery has been found to be involved [7, 8].
Although the treatment of BD is controversial, reconstruction with a prosthetic graft is usually unavoidable for an aneurysm or pseudoaneurysm, especially when the aorta is affected. Surgery must be performed as early as possible, because rupture of an inflammatory aneurysm can occur even when it is small. Because of the inflammation of the surrounding tissues, surgical repair is often difficult. Sutures tends to cut out, healing is often delayed and grafts are often affected by occlusion or by pseudoaneurysm formation. Arterial anastomoses should be performed only in disease-free segments, which means that intervention should be avoided in the active stage of the disease, if possible. Wrapping the anastomotic sites in a supplementary prosthetic sheet is advised in order to prevent pseudoaneurysm formation. Some authors prefer to perform only an aneurysmorraphy. Recently, Tuzun et al. [9] reported satisfactory results with tube graft insertion.
Apart from surgical intervention, many other therapeutic strategies have been tried with varying success. The use of anticoagulants does not always prevent occlusion or thrombosis. Postoperative corticosteroids are necessary to prevent arterial relapse. A combination of corticosteroids and immunosuppressive therapy (cyclophosphamide, azathioprine) has been found to be more effective than corticosteroids alone [10].
Notes
Correspondence to: A. El Maghraoui. 
References
- Roeyen G, Van Schil PEY, Vanmaele RGMTJ et al. Abdominal aortic aneurysm with lumbar vertebral erosion in Behçet's disease. A case report and review of the literature. Eur J Endovasc Surg1997;13:242–6.
- Mounsey JPD. Clinicopathological conference. Orogenital ulceration with phlebothrombosis (Behçet's syndrome) complicated by osteomyelitis of the lumbar spine and ruptured aorta. Br Med J1965;1:357–61.[Medline]
- Wang K, Hodges M. Erosion of lumbar vertebral bodies due to abdominal aortic aneurysm. Circulation1994;89:1317.
[Free Full Text] - International Study Group for Behçet's disease. Criteria of diagnosis of Behçet's disease. Lancet1990;335:1078–80.[Web of Science][Medline]
- Koç Y, Güllü I, Akpek G et al. Vascular involvement in Behçet's disease. J Rheumatol1992;19:402–10.[Web of Science][Medline]
- Lie JT. Vascular involvement in Behçet's disease: arterial and venous and vessels of all sizes. [Editorial]. J Rheumatol1992;19:341–2.[Medline]
- Kuzu MA, Özaslan C, Köksoy C, Gürler A, Tuzüner A. Vascular involvement in Behçet's disease: 8-year audit. World J Surg1994;18:948–54.[Web of Science][Medline]
- Hamza M. Large artery involvement in Behçet's disease. J Rheumatol1987;14:554–9.[Medline]
- Tuzun H, Besirli K, Sayin A et al. Management of aneurysms in Behçet's syndrome: an analysis of 24 patients. Surgery1997;121:150–6.[Web of Science][Medline]
- Le Thi Huong D, Wechsler B, Papo T et al. Arterial lesions in Behçet's disease. A study in 25 patients. J Rheumatol1995;22:2103–13.[Web of Science][Medline]
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