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[Arterial manifestations of Behçet's disease. 12 cases]
B Wechsler1, L T Lê Thi Huong Du, C de Gennes
1Service de médecine interne, groupe hospitalier Pitié-Salpêtrière, Paris.
Insights
Behçet's disease can cause serious arterial lesions, including aneurysms and stenosis, in a small percentage of patients. These vascular complications often require surgery and long-term monitoring due to poor response to medical treatment.
Area of Science:
- Vascular Medicine
- Rheumatology
- Immunology
Background:
- Behçet's disease is a multisystem inflammatory disorder.
- Non-coronary arterial lesions are a known, though less common, manifestation.
Observation:
- 12 out of 196 Behçet's disease patients (6.1%) presented with non-coronary arterial lesions.
- Lesions included stenosis, occlusion, arteriovenous fistula, and aneurysms (including anastomotic and false aneurysms).
- Histology revealed media fragmentation and vasculitis of vasa vasorum in some cases.
Findings:
- Arterial lesions appeared years after initial Behçet's disease diagnosis.
- Pulmonary aneurysms led to fatal hemoptysis in two patients.
- Combined corticosteroid and cyclophosphamide therapy was ineffective in preventing aneurysm development in two patients.
- Phlebitis co-occurred with arterial disease in 7 patients.
- No significant differences were found in disease onset, sex, clinical features, or HLA B5 presence between patients with and without arterial lesions.
Implications:
- Aneurysmal lesions in Behçet's disease have a poor response to medical management, necessitating surgical intervention.
- Recurrence of lesions at anastomosis sites highlights the need for prolonged patient monitoring post-surgery.
- Understanding these vascular complications is crucial for comprehensive Behçet's disease management.
Abstract:
Out of 196 patients with Behçet's disease, 12 (10 men and 2 women, mean age 34 +/- 7 years) had non-coronary arterial lesions. Behçet's disease was complete in 4 patients. The arterial lesions had appeared 8.6 +/- 8 years on average (20 years at most) after the first sign of the disease. Three patients showed evidence of stenosis or occlusion involving one or several arteries. Eight patients had both stenotic and aneurysmal lesions. One patient had an arteriovenous fistula. Another developed a false aneurysm at the site of introduction of a femoral catheter. Yet another patient developed an anastomotic aneurysm one year after implantation of an abdominal aortic graft. In 2 cases histology showed fragmentation of the media associated with vasculitis of the vasa vasorum. Two patients with pulmonary aneurysm died of massive haemoptysis. In 2 patients combined corticosteroid and cyclophosphamide therapy failed to prevent the development of aneurysmal lesions. Phlebitis was associated with arterial involvement in 7 patients. Comparison between patients with or without arterial lesions showed no significant difference in time of onset of Behçet's disease, sex, main clinical features and presence of HLA B5. Aneurysmal lesions respond poorly to medical treatment, and surgery is mandatory. Since recurrence at the site of anastomosis is possible, prolonged monitoring is required.