抗中心粒细胞胞浆抗体检测在儿科疾病中的应用

doi:10.12372/jcp.2022.22e0962

摘要/Abstract

摘要：

抗中性粒细胞胞浆抗体(ANCA)是一类以中性粒细胞和单核细胞胞浆成分为靶抗原的自身抗体。ANCA包括胞浆型(c-ANCA)、核周型(p-ANCA)和非典型(a-ANCA)。其中c-ANCA、p-ANCA的靶抗原分别是蛋白酶3和髓过氧化物酶。目前ANCA检测技术主要包括间接免疫荧光法、酶联免疫吸附法、化学发光法和荧光酶免疫法等。ANCA相关性血管炎是一种主要累及中小血管的坏死性血管炎,寡或无免疫复合物沉积,儿童发病率低,但起病急,进展快,临床表现多样化,停药后疾病易复发。ANCA已成为诊断儿童肉芽肿性血管炎、显微镜下血管炎和嗜酸性粒细胞肉芽肿性血管炎等系统性血管炎的重要标记物。ANCA检测有助于ANCA相关性血管炎的早期诊治,从而改善疾病预后。

关键词: 抗中心粒细胞胞浆抗体, 蛋白酶3, 髓过氧化物酶, ANCA相关性血管炎

Abstract:

Anti-neutrophil cytoplasmic antibodies (ANCA) are a class of autoantibodies that target the cytoplasmic components of neutrophils and monocytes. ANCA includes cytoplasmic ANCA (c-ANCA), perinuclear ANCA (p-ANCA) and atypical ANCA (a-ANCA). The target antigens of c-ANCA and p-ANCA are proteinase 3 and myeloperoxidase, respectively. At present, the ANCA detection assay mainly includes indirect immunofluorescence, enzyme-linked immunosorbent assay, chemiluminescent immunoassays and fluorescent-enzyme immunoassays. ANCA associated vasculitis (AAV) is a necrotizing vasculitis mainly involving small and medium sized arteries with pauci-immune deposits. The incidence of AAV is low in children, but the onset of the disease is urgent, the progression is rapid, the clinical manifestations are diverse, and the disease is easy to relapse after drug withdrawal. ANCA has become an important marker for the diagnosis of granulomatous vasculitis, microscopic vasculitis and eosinophilic granulomatous vasculitis. ANCA detection is helpful for the early diagnosis and treatment of AAV, thus improving the prognosis of the disease.

Key words: anti-neutrophil cytoplasmic antibody, proteinase 3, myeloperoxidase, ANCA-associated vasculitis

康郁林. 抗中心粒细胞胞浆抗体检测在儿科疾病中的应用[J]. 临床儿科杂志, 2022, 40(10): 733-738.

KANG Yulin. The application of anti-neutrophil cytoplasmic antibodies tests in pediatric diseases[J]. Journal of Clinical Pediatrics, 2022, 40(10): 733-738.

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疾病名称	分类标准
GPA	临床标准鼻血、溃疡、结痂、充血或堵塞,或鼻中隔缺损/穿孔：+3分;软骨受累：+2分;传导性或感音神经性听力受损：+1分
	实验室、影像和活检标准 c-ANCA或PR3阳性：+5分;胸部影像学：肺结节、包块或空洞：+2分;活检发现肉芽肿、血管外肉芽肿性炎症或巨细胞：+2分;影像学检查发现：鼻窦/副鼻窦炎症、实变或积液,或乳突炎：+1分;活检：寡免疫复合物肾小球肾炎：+1分;p-ANCA或MPO阳性：–1分;血嗜酸性粒细胞计数≥1×10⁹/L：–4分
	确诊标准当确诊为小或者中血管炎,且排除其他诊断。上述10项内容,总得分≥5分可诊断为GPA^[17]
EGPA	临床标准阻塞性气道疾病：+3分;鼻息肉：+3分;多发性单神经根炎：+1分
	实验室、影像和活检标准血嗜酸性粒细胞计数≥1×10⁹/L：+5分;活检可见血管外有嗜酸性粒细胞为主的浸润：+2分;c-ANCA或PR3阳性：–3分;血尿：–1分
	当确诊为小或者中血管炎,且排除其他诊断。上述7项条目,若总得分≥6分的患者,可诊断为EGPA^[5]
MPA	临床标准鼻腔出血、溃疡、结痂、充血或者鼻塞,或鼻中隔缺损/穿孔：–3分
	实验室、影像和活检标准 p-ANCA或MPO阳性：+6分;胸部影像学检查发现：纤维化或间质性肺病：+3分;活检可见寡免疫复合物肾小球肾炎：+3分;c-ANCA或PR3阳性：–1分;血嗜酸性粒细胞计数≥1×10⁹/L：–4分
	在排除类似血管炎的情况后,诊断为小血管或中血管炎的患者,上述6项内容,若总得分≥5分的患者,可诊断为MPA^[6]