Mareen P, Van De Walle S, Bernaert P, Vanhouteghem H, Dierick J
Ziekenhuis Maria Middelares Sint-Jozef, Kortrijksesteenweg 1026, 9000 Gent.
Acta Clin Belg. 2003 May-Jun;58(3):193-200. doi: 10.1179/acb.2003.58.3.008.
We report on one patient with Wegener's granulomatosis (WG) and two patients with microscopic polyangiitis (MPA). The patient with WG had signs of a respiratory infection and showed a c-ANCA pattern with proteinase 3 (PR3) specificity. The patients with MPA presented with pulmonary haemorrhage and signs of renal damage and showed a p-ANCA pattern with myeloperoxidase (MPO) specificity. In the three patients histopathological findings confirmed the diagnosis. We discuss the clinical indications of ANCA testing and the current terminology for reporting ANCA results (c-ANCA, p-ANCA, c-ANCA (atypical) and atypical ANCA). The target antigens and diseases associated with these different patterns are considered. Finally we focus on the value of ANCA and more specific PR3-ANCA and MPO-ANCA in the diagnosis of WG and MPA. The new application domain of ANCA in Crohn's disease and ulcerative colitis is also discussed.
我们报告了1例韦格纳肉芽肿（WG）患者和2例显微镜下多血管炎（MPA）患者。WG患者有呼吸道感染体征，且抗中性粒细胞胞浆抗体（ANCA）呈胞浆型（c-ANCA），具有蛋白酶3（PR3）特异性。MPA患者表现为肺出血和肾损害体征，且ANCA呈核周型（p-ANCA），具有髓过氧化物酶（MPO）特异性。在这3例患者中，组织病理学检查结果证实了诊断。我们讨论了ANCA检测的临床指征以及报告ANCA结果的当前术语（c-ANCA、p-ANCA、非典型c-ANCA和非典型ANCA）。还考虑了与这些不同模式相关的靶抗原和疾病。最后，我们重点关注ANCA以及更具特异性的PR3-ANCA和MPO-ANCA在WG和MPA诊断中的价值。还讨论了ANCA在克罗恩病和溃疡性结肠炎中的新应用领域。
