Juvenile arteritis revisited. Buerger's disease-Takayasu's disease

Abstract

Thromboangiitis obliterans is defined as an inflammatory disease involving small and medium sized arteries and veins. As no clinical symptoms, signs, radiological or laboratory abnormalities nor pathological features are specific, the diagnosis is usually made according the criteria proposed by Adar in a young smoker, male or female, with distal arterial occlusions sometimes associated with thrombophlebitis and with negative aetiological investigations. Recently, rheumatic manifestations were identified in about 10% of patients. If new developments in vascular medicine, as ultrasound explorations or CT scan limit the indications for angiography, others, especially in the field of thrombophilia, make more difficult the differential diagnosis. If the strong link between the disease activity and smoking habits is confirmed, medical or surgical approach remain disappointing. Takayasu's disease or non specific aorto-arteritis is an inflammatory disease of young women that primarily affects the aorta its main branches and the pulmonary arteries. The disease often start in children. The prevalence of the disease as the arterial lesions show geographical variations. That and the different associations with infectious, rheumatic or systemic diseases could justify to consider Takayasu's disease as a syndrome. B-mode ultrasonography and electron beam computed tomography are usually sufficient to characterize the arterial involvement and are essential for the follow up of patients. In some rare cases arterial biopsy is necessary to confirm the diagnosis. The surgical treatment concerns specific lesions with ischemic symptoms and failure of the medical approach, however endovascular techniques are often discussed in first intention. During inflammatory phases with evolutive lesions corticosteroids are often used. In case of failure cyclophosphamide or methotrexate have been used.