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巨细胞动脉炎

Giant cell arteritis.

作者信息

Levine Stuart M, Hellmann David B

机构信息

Division of Molecular and Clinical Rheumatology, Johns Hopkins University School of Medicine, Baltimore, Maryland 21224, USA.

出版信息

Curr Opin Rheumatol. 2002 Jan;14(1):3-10. doi: 10.1097/00002281-200201000-00002.

Abstract

Giant cell arteritis (GCA), the most common form of systemic vasculitis in adults, preferentially involves large and medium-sized arteries in patients over the age of 50. The classic manifestations are headache, jaw claudication, polymyalgia rheumatica (PMR), and visual symptoms, but 40% of patients present with a wide range of occult manifestations. Early diagnosis and treatment with prednisone can prevent blindness, the most feared complication of GCA. The pathogenesis of GCA is T-cell dependent and antigen driven. Clinical subsets of GCA appear to result from variable cytokine expression. The risk of developing thoracic aortic aneurysm is increased more than 17-fold in patients with GCA. GCA can also involve large arteries, especially the subclavian and axillary arteries. Color Doppler ultrasound, magnetic resonance imaging, and positron-emission tomography scanning are providing insights into the extent and pathogenesis of the disease but have not replaced temporal artery biopsy as the gold standard for securing the diagnosis. Two recently completed double-blind, placebo-controlled trials concerning whether methotrexate plus prednisone is more effective than prednisone alone reached conflicting conclusions.

摘要

巨细胞动脉炎(GCA)是成人系统性血管炎最常见的形式,主要累及50岁以上患者的大中型动脉。其典型表现为头痛、颌部间歇性运动障碍、风湿性多肌痛(PMR)和视觉症状,但40%的患者表现出广泛的隐匿性症状。早期使用泼尼松进行诊断和治疗可预防失明,这是GCA最可怕的并发症。GCA的发病机制是T细胞依赖性和抗原驱动的。GCA的临床亚组似乎是由细胞因子表达的差异导致的。GCA患者发生胸主动脉瘤的风险增加了17倍以上。GCA也可累及大动脉,尤其是锁骨下动脉和腋动脉。彩色多普勒超声、磁共振成像和正电子发射断层扫描有助于了解该病的范围和发病机制,但尚未取代颞动脉活检作为确诊的金标准。最近完成的两项关于甲氨蝶呤加泼尼松是否比单独使用泼尼松更有效的双盲、安慰剂对照试验得出了相互矛盾的结论。

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