抗中性粒细胞胞浆抗体在变应性肉芽肿性血管炎中的患病率及临床意义。

Prevalence and clinical significance of antineutrophil cytoplasmic antibodies in Churg-Strauss syndrome.

作者信息

Sinico Renato A, Di Toma Lucafrancesco, Maggiore Umberto, Bottero Paolo, Radice Antonella, Tosoni Cinzia, Grasselli Chiara, Pavone Laura, Gregorini Gina, Monti Stefano, Frassi Micol, Vecchio Filomena, Corace Caterina, Venegoni Emanuela, Buzio Carlo

机构信息

Dipartimento di Nefrologia e Immunologia, Azienda Ospedaliera Ospedale San Carlo Borromeo, Milan, Italy.

出版信息

Arthritis Rheum. 2005 Sep;52(9):2926-35. doi: 10.1002/art.21250.

DOI:10.1002/art.21250

PMID:16142760

Abstract

OBJECTIVE

Churg-Strauss syndrome (CSS) is classified among the so-called antineutrophil cytoplasmic antibody-associated systemic vasculitides (AASVs) because of its clinicopathologic features that overlap with the other AASVs. However, while antineutrophil cytoplasmic antibodies (ANCAs) are consistently found in 75-95% of patients with Wegener's granulomatosis or microscopic polyangiitis, their prevalence in CSS varies widely and their clinical significance remains uncertain. We undertook this study to examine the prevalence and antigen specificity of ANCAs in a large cohort of patients with CSS. Moreover, we evaluated the relationship between ANCA positivity and clinicopathologic features.

METHODS

Immunofluorescence and enzyme-linked immunosorbent assay were used to determine the presence or absence of ANCAs in 93 consecutive patients at the time of diagnosis. The main clinical and pathologic data, obtained by retrospective analysis, were correlated with ANCA status.

RESULTS

ANCAs were present by immunofluorescence in 35 of 93 patients (37.6%). A perinuclear ANCA (pANCA) pattern was found in 26 of 35 patients (74.3%), with specificity for myeloperoxidase (MPO) in 24 patients, while a cytoplasmic ANCA pattern, with specificity for proteinase 3, was found in 3 of 35 patients (8.6%). Atypical patterns were found in 6 of 30 patients with anti-MPO antibodies (20.0%). ANCA positivity was associated with higher prevalences of renal disease (51.4% versus 12.1%; P < 0.001) and pulmonary hemorrhage (20.0% versus 0.0%; P = 0.001) and, to a lesser extent, with other organ system manifestations (purpura and mononeuritis multiplex), but with lower frequencies of lung disease (34.3% versus 60.3%; P = 0.019) and heart disease (5.7% versus 22.4%; P = 0.042).

CONCLUSION

ANCAs are present in approximately 40% of patients with CSS. A pANCA pattern with specificity for MPO is found in most ANCA-positive patients. ANCA positivity is mainly associated with glomerular and alveolar capillaritis.

摘要

目的

变应性肉芽肿性血管炎（CSS）因其临床病理特征与其他抗中性粒细胞胞浆抗体相关系统性血管炎（AASV）重叠，而被归类于AASV。然而，虽然在75% - 95%的韦格纳肉芽肿或显微镜下多血管炎患者中始终能检测到抗中性粒细胞胞浆抗体（ANCA），但其在CSS患者中的患病率差异很大，临床意义仍不明确。我们开展这项研究以检测一大群CSS患者中ANCA的患病率及抗原特异性。此外，我们评估了ANCA阳性与临床病理特征之间的关系。

方法

采用免疫荧光法和酶联免疫吸附测定法，在93例连续诊断的患者确诊时检测ANCA的有无。通过回顾性分析获得的主要临床和病理数据与ANCA状态相关联。

结果

93例患者中有35例（37.6%）免疫荧光显示ANCA阳性。35例患者中有26例（74.3%）呈现核周型ANCA（pANCA）模式，其中24例对髓过氧化物酶（MPO）具有特异性；35例患者中有3例（8.6%）呈现胞浆型ANCA模式，对蛋白酶3具有特异性。30例抗MPO抗体阳性患者中有6例（20.0%）呈现非典型模式。ANCA阳性与肾脏疾病（51.4% 对12.1%；P < 0.001）和肺出血（20.0% 对0.0%；P = 0.001）的较高患病率相关，在较小程度上与其他器官系统表现（紫癜和多发性单神经炎）相关，但与肺部疾病（34.3% 对60.3%；P = 0.019）和心脏病（5.7% 对22.4%；P = 0.042）的较低发生率相关。