ANCA-positive periaortic vasculitis: does it fall within the spectrum of vasculitis?
ANCA-positive periaortic vasculitis: does it fall within the spectrum of vasculitis?
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DOI：
10.1046/j.1365-2796.2002.00948.x
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发表时间：
2002-03-01
影响因子：
11.1
通讯作者：
Buzio, C
中科院分区：
文献类型：
--
作者：
Vaglio, A;Manenti, L;Buzio, C
腹膜后纤维化（RPF）是一种未知的活动机制的疾病，有时报告与结缔组织疾病和系统性血管炎。我们在此报告两例抗神经细胞胞浆抗体（ANCA）阳性的RPF显示临床证据的快速进展性肾小球肾炎。虽然泼尼松和环磷酰胺治疗导致RPF缓解在这两种情况下，肾功能恢复，只有一个病人和其他进展为慢性肾功能衰竭。本文综述了ANCA阳性RPF的相关文献，并对ANCA阳性小血管炎与RPF的关系进行了讨论。
Retroperitoneal fibrosis (RPF) is a disease of unknown actiology that has sometimes been reported in association with connective tissue disorders and systemic vasculitis. We report here two cases of antineutrophil cytoplasmic antibody (ANCA)-positive RPF showing clinical evidence of rapidly progressive glomerulonephritis. Although treatment with prednisone and cyclophosphamide led to a remission of RPF in both cases, renal function was restored in only one patient and the other progressed to chronic renal failure. The paper reviews the literature concerning ANCA-positive RPF and discusses the relationship between ANCA-positive vasculitis and RPF.