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本月疾病。变应性肉芽肿性血管炎。

Disease of the month. The Churg Strauss Syndrome.

作者信息

Eustace J A, Nadasdy T, Choi M

机构信息

Division of Nephrology, Johns Hopkins University School of Medicine, Baltimore, Maryland 21205, USA.

出版信息

J Am Soc Nephrol. 1999 Sep;10(9):2048-55. doi: 10.1681/ASN.V1092048.

Abstract

The Churg Strauss Syndrome is an eosinophil-associated small vessel vasculitis. Although its pathogenesis may be distinctive and the association with severe late-onset asthma typical, the clinical features during the vasculitic phase widely overlap with those of the other forms of necrotizing vasculitis, and no single clinical or histologic feature is pathognomic of the condition. Renal involvement is common, although usually mild, and even when severe it tends to respond well to treatment. The prognosis for both patient and renal survival with adequate treatment is in general good. The optimal treatment strategy, however, is uncertain, and may differ from that for the other vasculitides. In particular, in contrast to Wegener's granulomatosis, the need for routine cyclophosphamide treatment is unconfirmed and requires further study.

摘要

变应性肉芽肿性血管炎是一种与嗜酸性粒细胞相关的小血管血管炎。尽管其发病机制可能独特,且与严重迟发性哮喘的关联较为典型,但血管炎阶段的临床特征与其他形式的坏死性血管炎广泛重叠,没有单一的临床或组织学特征对该疾病具有诊断意义。肾脏受累很常见,尽管通常较轻,即使严重时对治疗也往往反应良好。经过充分治疗,患者和肾脏存活的预后总体良好。然而,最佳治疗策略尚不确定,可能与其他血管炎不同。特别是,与韦格纳肉芽肿病相比,常规环磷酰胺治疗的必要性尚未得到证实,需要进一步研究。

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