The Unique Features of Vasculitis in Behçet’s Syndrome

Abstract

The presence of a true vasculitis is difficult to discern in some of the more common manifestations of Behçet's syndrome, like the papulopustular lesions of the skin. On the other hand, a true vasculitis is seen in all vessel sizes in the majority of the patients. The pathogenesis is not yet known. A Th1-type inflammatory reaction is seen like in some other primary vasculitides. However, in contrast to other vasculitides, granuloma formation is absent. Behçet's syndrome is unique among the vasculitides with its differing geographic distribution in disease expression, the distinctly more severe disease among the male, the predominance of venous disease, and the generally abating disease course with lack of associated increased atherosclerosis.

Vascular Manifestations of Behçet’s Disease

“…Reports from Turkey and Korea described the femoral artery and the abdominal aorta as the most common locations of aneurysm formation [49,50]. However, a report from a Chinese registry showed the lower extremity and the abdominal aorta as the most frequently attacked sites and the femoral artery as a rarely attacked location [47].…”

Aortic thrombosis in a patient with a late diagnosis of Behcet's disease

“…It is characterized by recurrent oral ulcers and systemic manifestations including genital ulcers, cutaneous lesions, arthropathy and ocular, neurological or vascular disease. [1][2][3][4][5][6] Vascular involvement occurs in 23 to 50% of patients and is more common among male patients and young patients, with onset commonly occurring later on in the course of the disease.…”

“…7 Vascular involvement of major vessels occurs in up to 35 to 39% of patients, 5,14 typically involving pulmonary arteries, the aorta, the iliac arteries, femoral arteries or popliteal arteries. 6,14 This is one of the few manifestations of the disease that are associated with systemic symptoms and laboratory evidence 7 and, in general (33%), occurs during the first 5 years after diagnosis. 8,12 However, in 7 to 30% of patients, vascular involvement has taken place before the disease is diagnosed clinically.…”

“…8,12 However, in 7 to 30% of patients, vascular involvement has taken place before the disease is diagnosed clinically. 6,8 In terms of treatment, the indications for surgery are the same as followed for peripheral arterial occlusive disease of other etiologies. However, it is necessary to conduct rigorous monitoring of both vascular and rheumatological aspects because of the increased risks of disease recurrence, of graft thrombosis and of anastomotic aneurysm.…”

Immunopathogenesis Based Adamantiades- Behcet Disease Vasculitis Treatment

“…The term 'vasculo-Behcet' has been adopted to describe cases where vascular features dominate in disease expression. After initial vascular lesion (usually venous thrombosis), other vascular lesions may follow and lead to progressive, multifocal vascular disease with an unfavorable prognosis (Melikoglu et al, 2008). Vascular inflammation is mainly due to complex innate-immune, pro-inflammatory mechanisms which activate and perpetuate adaptive/specific immune responses against antigens not identified clearly.…”