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“丝绸之路病”(白塞病)

'Silk route disease' (Behçet's disease).

作者信息

James D G

出版信息

West J Med. 1988 Apr;148(4):433-7.

Abstract

Behçet's disease is a multisystem disorder in which orogenital ulceration is associated with troublesome generalized uveitis, erythema nodosum, pyoderma, dermatographism, seronegative arthritis, and neurologic and cardiovascular symptoms. There is no diagnostic laboratory test; the diagnosis is based on the disorder's multisystem clinical features. A points scoring system is helpful in distinguishing it from other multisystem disorders that mimic it. It occurs most frequently in an area coinciding with the old Silk Route, between latitudes 30 degrees and 45 degrees north, in Asian and Eurasian populations, and it has an HLA-B51 affinity. The cause remains unknown, but a postulated trigger factor is a herpesvirus with cofactors that include ethnic group, human leukocyte antigen affinities, T-cell and autonomic imbalance, circulating immune complexes, autoimmunity, blood viscosity, decreased fibrinolysis, and zinc deficiency. Treatment includes administering corticosteroids, azathioprine, chlorambucil, cyclosporine, and colchicine, and fibrinolytic therapy.

摘要

白塞病是一种多系统疾病,其口生殖器溃疡与严重的全身性葡萄膜炎、结节性红斑、脓皮病、皮肤划痕症、血清阴性关节炎以及神经和心血管症状相关。目前尚无诊断性实验室检查;诊断基于该疾病的多系统临床特征。积分系统有助于将其与其他类似的多系统疾病区分开来。它最常发生在北纬30度至45度之间与古代丝绸之路重合的地区,多见于亚洲和欧亚人群,且与HLA - B51相关。病因尚不清楚,但推测触发因素是一种疱疹病毒,其辅助因素包括种族、人类白细胞抗原亲和力、T细胞和自主神经失衡、循环免疫复合物、自身免疫、血液粘度、纤维蛋白溶解降低以及锌缺乏。治疗方法包括使用皮质类固醇、硫唑嘌呤、苯丁酸氮芥、环孢素和秋水仙碱,以及纤维蛋白溶解疗法。

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本文引用的文献

1
Behçet's disease.
Ophthalmology. 1982 Nov;89(11):1279-84. doi: 10.1016/s0161-6420(82)34644-8.
2
Behçet's disease.
Trans Ophthalmol Soc U K (1962). 1982 Apr;102 (Pt 1):174-7.
3
Close association of HLA-Bw51 with Behçet's disease.HLA - Bw51与白塞病的密切关联。
Arch Ophthalmol. 1982 Sep;100(9):1455-8. doi: 10.1001/archopht.1982.01030040433013.
4
Behçet's disease and close contact with pigs.
Acta Med Scand. 1984;216(5):541-3. doi: 10.1111/j.0954-6820.1984.tb05044.x.

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