98例患者中抗中性粒细胞胞浆抗体（ANCA）的临床意义

Clinical significance of ANCA in 98 patients.

作者信息

Geffriaud-Ricouard C, Noël L H, Chauveau D, Houhou S, Grünfeld J P, Lesavre P

机构信息

Département de Néphrologie, Hôpital Necker, Paris, France.

出版信息

Adv Exp Med Biol. 1993;336:273-9. doi: 10.1007/978-1-4757-9182-2_41.

DOI:10.1007/978-1-4757-9182-2_41

PMID:7905232

Abstract

Clinical and histological data leading to precise diagnosis were retrospectively obtained in 98 patients with antineutrophil cytoplasmic antibodies (ANCA) detected by indirect immunofluorescence (IIF). Specificity was determined by myeloperoxidase (MPO) and proteinase 3 (PR3) specific ELISA in all and a comparative study based on ANCA specificity was performed. Vasculitis was present in all cases. PR3-ANCA occurred predominantly in males (25/38) with WG (19/38). MPO-ANCA occurred predominantly in older women and were often associated with various autoimmune disorders. There was a high prevalence of lung hemorrhage (18/45) and mPA (26/45) in this group. Patients with negative MPO and PR3 specific ELISA despite positive IIF (n = 15) were almost exclusively WG (13/15) and were characterized by a high prevalence of hepatic and digestive manifestations. Renal and patient survival at the 75th percentile was 15 months with MPO-ANCA and 16 months with PR3, and was similar for patients with WG and mPA. With immunosuppressive treatment, ANCA disappeared in 66% of cases and this disappearance was always associated with absence of disease activity.

摘要

通过间接免疫荧光法（IIF）检测出抗中性粒细胞胞浆抗体（ANCA）的98例患者，回顾性获取了有助于精确诊断的临床和组织学数据。所有患者均通过髓过氧化物酶（MPO）和蛋白酶3（PR3）特异性酶联免疫吸附测定（ELISA）确定特异性，并基于ANCA特异性进行了一项对比研究。所有病例均存在血管炎。PR3-ANCA主要见于男性（25/38），与韦格纳肉芽肿（WG，19/38）相关。MPO-ANCA主要见于老年女性，常与各种自身免疫性疾病相关。该组中肺出血（18/45）和显微镜下多血管炎（mPA，26/45）的患病率较高。尽管IIF呈阳性，但MPO和PR3特异性ELISA阴性的患者（n = 15）几乎均为WG（13/15），其特征为肝脏和消化系统表现的患病率较高。MPO-ANCA患者第75百分位数的肾脏和患者生存率为15个月，PR3患者为16个月，WG和mPA患者的生存率相似。经过免疫抑制治疗，66%的病例中ANCA消失，且这种消失总是与疾病活动的消失相关。