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变应性肉芽肿性血管炎:最新进展。

Churg-strauss syndrome: an update.

机构信息

Mayo College of Medicine, Mayo Clinic Florida, Jacksonville, FL 32224, USA.

出版信息

Curr Rheumatol Rep. 2011 Dec;13(6):489-95. doi: 10.1007/s11926-011-0205-7.

Abstract

Churg-Strauss syndrome is an uncommon disease of unknown cause described initially by Churg and Strauss in 1951. Even though it was initially thought to be a variant of polyarteritis nodosa, its pathological, clinical, and laboratory features show that it is related to the small vessel vasculitides, and it is now classified as an antineutrophil cytoplasmic antibody-associated vasculitis. The presence of asthma, usually of adult onset, along with other allergic symptoms, peripheral and tissue eosinophilia, is specific to this disease. These features usually help clinicians distinguish it from other types of small vessel vasculitis and should alert clinicians about its presence. Two different clinical subtypes defined by the presence of antineutrophil cytoplasmic antibodies recently have been recognized. Recent advances in the treatment and pathophysiology of Churg-Strauss syndrome are reviewed in this article.

摘要

变应性肉芽肿性血管炎是一种病因不明的罕见疾病,最初由 Churg 和 Strauss 于 1951 年描述。尽管最初认为它是结节性多动脉炎的一种变异型,但它的病理学、临床和实验室特征表明它与小血管血管炎有关,现在被归类为抗中性粒细胞胞质抗体相关性血管炎。哮喘的存在,通常为成人发病,以及其他过敏症状、外周和组织嗜酸性粒细胞增多,是该疾病的特异性表现。这些特征通常有助于临床医生将其与其他类型的小血管血管炎区分开来,并提醒临床医生注意其存在。最近,根据抗中性粒细胞胞质抗体的存在,已经确定了两种不同的临床亚型。本文综述了变应性肉芽肿性血管炎的治疗和病理生理学的最新进展。

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