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21世纪的血栓闭塞性脉管炎:诊断、临床特征及治疗

Buerger's disease in the 21st century: diagnosis, clinical features, and therapy.

作者信息

Mills Joseph L

机构信息

Division of Vascular Surgery, University of Arizona Health Sciences Center, Tucson, AZ, USA.

出版信息

Semin Vasc Surg. 2003 Sep;16(3):179-89. doi: 10.1016/s0895-7967(03)00023-1.

DOI:10.1016/s0895-7967(03)00023-1
PMID:12975757
Abstract

Buerger's disease (thromboangiitis obliterans or TAO) is a clinical syndrome characterized by the development of segmental thrombotic occlusions of the medium and small arteries of the extremities. It is clinically and pathologically distinguishable from arteriosclerosis and necrotizing arteritis. Afflicted patients are mostly young, male, inveterate tobacco smokers who present with distal extremity ischemia, ischemic ulcers, or frank gangrene of the toes or fingers. Large arteries are typically spared, as are the coronary, cerebral, and visceral circulations. While mortality is not increased, patients with Buerger's disease often suffer from severe ischemic pain and tissue loss culminating in minor and major limb amputation. Clinical diagnostic criteria generally include the following: (1) history of smoking or tobacco abuse; (2) age of onset less than 45 to 50 years; (3) infrapopliteal, segmental arterial occlusions with sparing of the proximal vasculature; (4) frequent distal upper extremity arterial involvement (Raynaud's syndrome or digital ulceration); (5) superficial phlebitis; and (6) exclusion of arteriosclerosis, diabetes, true arteritis, proximal embolic source, and hypercoagulable states. Typical arteriographic patterns have been described that are suggestive, but not pathognomonic. While the cause of Buerger's disease remains unknown, the disease onset and clinical course are inextricably linked to tobacco abuse. Acute Buerger's disease is characterized histopathologically by intensely cellular vessel wall inflammation, giant cell foci, and hypercellular thrombi, but with preservation of the elastic lamina and the overall vascular wall architecture. Most investigators feel that Buerger's disease is an immune-mediated endarteritis; recent immunocytochemical studies have identified the linear deposition of immunoglobulins and complement factors along the elastic lamina. The inciting antigen has not been discovered. Tobacco abstinence generally results in disease quiescence and remains the mainstay of treatment.

摘要

血栓闭塞性脉管炎(伯格氏病或血栓性血管炎闭塞症,即TAO)是一种临床综合征,其特征为四肢中小动脉节段性血栓性闭塞。在临床和病理上,它与动脉硬化和坏死性动脉炎可相鉴别。患病患者大多为年轻男性,有长期吸烟习惯,表现为远端肢体缺血、缺血性溃疡或足趾或手指明显坏疽。大动脉通常不受累,冠状动脉、脑循环和内脏循环也是如此。虽然死亡率没有增加,但伯格氏病患者常遭受严重的缺血性疼痛和组织缺失,最终导致肢体大小截肢。临床诊断标准通常包括以下几点:(1)吸烟或有烟草滥用史;(2)发病年龄小于45至50岁;(3)腘动脉以下节段性动脉闭塞,近端血管未受累;(4)上肢远端动脉频繁受累(雷诺综合征或手指溃疡);(5)浅表性静脉炎;(6)排除动脉硬化、糖尿病、真性动脉炎、近端栓子来源和高凝状态。已描述了典型的血管造影模式,这些模式具有提示性,但并非特异性。虽然伯格氏病的病因尚不清楚,但疾病的发作和临床过程与烟草滥用有着千丝万缕的联系。急性伯格氏病在组织病理学上的特征是血管壁有强烈的细胞炎症、巨细胞灶和细胞过多的血栓形成,但弹性膜和整个血管壁结构得以保留。大多数研究人员认为伯格氏病是一种免疫介导的动脉内膜炎;最近的免疫细胞化学研究已确定免疫球蛋白和补体因子沿弹性膜呈线性沉积。引发抗原尚未被发现。戒烟通常会使疾病静止,并仍然是治疗的主要手段。

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