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嗜酸性肉芽肿性多血管炎(变态性肉芽肿血管炎)的临床特征和诊断

Author
Talmadge E King, Jr, MD
Section Editors
Kevin R Flaherty, MD, MS
Richard J Glassock, MD, MACP
Bruce S Bochner, MD
Deputy Editor
Helen Hollingsworth, MD
Translators
李晓军, 主任技师，教授

引言

嗜酸性肉芽肿性多血管炎(eosinophilic granulomatosis with polyangiitis, EGPA)以前被称作变态性肉芽肿血管炎 (Churg-Strauss syndrome, CSS)或变应性肉芽肿性血管炎，是一种以慢性鼻-鼻窦炎、哮喘及显著外周血嗜酸性粒细胞增多为特征的多系统疾病[1-7]。EGPA被归类为中小动脉血管炎，但血管炎在该病初期常不明显。

最常受累的器官为肺，其次为皮肤。然而，EGPA可累及任何器官系统，包括心血管、胃肠道、肾脏和中枢神经系统。EGPA引起的并发症和死亡主要是由肺外器官血管炎导致的。

EGPA的临床特征和诊断将总结在此。EGPA的流行病学、发病机制、治疗和预后以及对存在血管炎和/或嗜酸性粒细胞增多患者的认识将单独讨论。 (参见“嗜酸性肉芽肿性血管炎(Churg-Strauss综合征)的流行病学、发病机制和病理学”和“Treatment and prognosis of eosinophilic granulomatosis with polyangiitis (Churg-Strauss)”和“成人血管炎的分类及诊断方法”和“对不明原因嗜酸性粒细胞增多患者的概述”)

临床特征

疾病分期 — EGPA的临床特征通常见于几个连续阶段，但这几个阶段并不总是可明显区分[8,9]：

●前驱期–前驱期发生于10-30岁的个体中，其特征为特应性疾病、变态反应性鼻炎和哮喘。

                         

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Literature review current through: 2017-06 . | This topic last updated: 2016-07-08.
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