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[变应性肉芽肿性血管炎:来自意大利单一中心的38例患者的结局及长期随访]

[Churg-Strauss syndrome: outcome and long-term follow-up of 38 patients from a single Italian centre].

作者信息

Baldini C, Della Rossa A, Grossi S, Catarsi E, Talarico R, d'Ascanio A, Mosca M, Neri R, Tavoni A, Bombardieri S

机构信息

U.O. Reumatologia, Università di Pisa, 56100 Pisa, Italia.

出版信息

Reumatismo. 2009 Apr-Jun;61(2):118-24. doi: 10.4081/reumatismo.2009.118.

Abstract

OBJECTIVE

This study was aimed at verifying any potential correlation between anti-myeloperoxidase antineutrophil cytoplasmic antibodies (ANCA-MPO) and clinical features and outcome indices in Churg-Strauss Syndrome (CSS).

METHODS

Thirty-eight Churg-Strauss syndrome patients were selected from the medical records of all vasculitis patients attending the Rheumatology and Immunology Unit at the Department of Internal Medicine of the University of Pisa in the decades between 1989 and 2008. Data were analysed retrospectively. Statistical analyses of the results were carried out using the Mann-Whitney test to determine the correlations between the clinical and serological parameters. Qualitative variables were compared using contingency table analysis and Fisher's exact test.

RESULTS

ANCA-MPO were detected in15/38 (39%) patients. Positive ANCA status was associated with peripheral neuropathy (p=0.0006), whereas negative ANCA status was associated with lung involvement (p=0.002). Relapses were strongly associated with positive ANCA status (p=0.01) and with an increase in- or a reappearance of ANCA-MPO levels (p=0.006). Finally, ANCA-MPO were significantly associated with neurological damage (p=0.003).

CONCLUSIONS

The presence or absence of ANCA-MPO identify different clinical subsets in CSS. Overall, ANCA-MPO appears as a useful tool in the monitoring of CSS and in particular a good predictor of CSS relapses.

摘要

目的

本研究旨在验证抗髓过氧化物酶抗中性粒细胞胞浆抗体(ANCA-MPO)与变应性肉芽肿性血管炎(CSS)的临床特征及预后指标之间是否存在潜在关联。

方法

从1989年至2008年期间比萨大学内科风湿病与免疫学单元诊治的所有血管炎患者病历中选取38例变应性肉芽肿性血管炎患者。对数据进行回顾性分析。采用曼-惠特尼检验对结果进行统计学分析,以确定临床和血清学参数之间的相关性。使用列联表分析和费舍尔精确检验对定性变量进行比较。

结果

在15/38(39%)例患者中检测到ANCA-MPO。ANCA阳性状态与周围神经病变相关(p = 0.0006),而ANCA阴性状态与肺部受累相关(p = 0.002)。复发与ANCA阳性状态密切相关(p = 0.01),也与ANCA-MPO水平升高或再次出现相关(p = 0.006)。最后,ANCA-MPO与神经损伤显著相关(p = 0.003)。

结论

ANCA-MPO的存在与否可识别CSS中的不同临床亚组。总体而言,ANCA-MPO似乎是监测CSS的有用工具,尤其是CSS复发情况的良好预测指标。

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