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68-5-第五节 血管炎的诊断与治疗
系统性血管炎的临床表现复杂多样且无特异性,故给临床诊断带来一定的困难。系统性血管炎需根据临床表现、实验室检查、病理活检资料以及影像学资料包括 X一线胸片、血管造影、CT, MRI等综合判断,以确定血管炎的类型及病变范围。如出现无法解释的下列情况时,应考虑血管炎的可能:①多系统损害。②进行性肾小球肾炎或血肌ffF和尿素氮进行性升高。③肺部多变阴影或固定的阴影/空洞。④多发性单神经根炎或多神经根炎。⑤不明原因发热。⑥缺血性或淤血性症状。⑦紫瘫性皮疹或网状青斑。⑧结节性坏死性皮疹。⑨无脉或血压升高。⑩不明原因的耳鼻喉或眼部病变。)ANCA,AECA阳性。此外,在作出血管炎诊断时应除外感染、肿瘤以及弥漫性结缔组织病,如系统性红斑狼疮、类风湿关节炎、干燥综合征等。
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+ _' D( o( P% y- O" x 血管炎的治疗原则是早期诊断、早期治疗,以防止不可逆的损害。患者一旦确诊为血管炎,就应积极治疗,即使尚未明确分型,以免因分型而延误病情,贻误治疗时机。血管炎的常用治疗药物为糖皮质激素和免疫抑制剂,后者以环磷酞胺最为常用。部分血管炎,如川崎病使用IVIg(静脉用丙种球蛋白)具有良好效果。其他还可试用血浆置换。近年来,不断地有新的生物制剂用于临床。抗感染治疗在韦格纳肉芽肿的治疗中具有作用。各种血管炎的具体治疗方法见各论。 $ h: W$ ^- l7 j, s, b* p1 R
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为了评估血管炎的病情活动情况,各国学者制定了不少评分标准,较常用的有系统性坏死性血管炎的伯明翰评分 (Birmingham vas-culitis activity score, BVAS) ,Olsen等制定的血管炎活动指数(Vasculitis activity index, VAI)和Kallenberg等制定的韦格纳肉芽肿的疾病活动评分标准(Criteria for disease activity in We-gener' s granulomatosis) o这些评分标准用于评估病情和预后,指导治疗。血管炎在合并感染以及并发其他疾病时,临床表现可以类似于病情活动,为区别于病情本身的活动,可采用BVAS进行评估,也具有指导意义。以下给出BVAS评分系统。
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伯明翰系统性血管炎活动评分(Birming-ham vasculitis activity score, BVAS):
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