Zhang Xuan, Dong Yi, Zeng Xiaofeng, Li Yongzhe, Tang Fulin
Department of Rheumatology, Peking Union Medical College Hospital, PUMC & CAMS, Beijing 100730.
Chin Med Sci J. 2002 Mar;17(1):32-5.
To compare the clinical and pathological manifestations of patients with antineutrophil cytoplasmic autoantibodies (ANCA) directed against proteinase 3 (anti-PR3) or myeloperoxidase (anti-MPO).
One hundred and forty patients with ANCA were detected for anti-PR3 and anti-MPO by ELISA. The clinical features at presentation, histopathological characteristics and outcome of all patients who were tested positive for anti-PR3 or anti-MPO were analysed.
In anti-PR3 group (n = 21), 16 cases (76.2%) had systemic vasculitis, in which Wegener's granulomatosis prevailed (13 cases, 61.9%). In anti-MPO group (n = 31), 19 cases (61.3%) were diagnosed as systemic vasculitis and 12 cases (38.7%) as microscopic angiitis. For vasculitic patients with anti-PR3 and anti-MPO, the disease duration at diagnosis was 9.6 +/- 2.0 m and 4.4 +/- 0.9 m respectively, P < 0.05; vasculitis activity index (BVAS) and mean number of affected organ were 22.5 +/- 2.1, 5.0 +/- 0.4 and 25.1 +/- 1.7, 4.8 +/- 0.4 respectively, P > 0.05; upper respiratory tract, eye and joint involvements were 11(68.8%), 7(43.8%), 11(68.8%) and 7(36.8%), 2(10.5%), 5(26.3%) respectively, P < 0.05. Although there was no statistical difference in renal involvement between these two groups, patients with serum creatine > 500 micromol/L were more commonly seen in anti-MPO group than in anti-PR3 group, which were 8(42.1%) and 2(12.5%) respectively, P < 0.05. Ten relapses were seen in anti-PR3 group and only 2 in anti-MPO group, but the acute mortality rate in anti-MPO group (5/19, 27.4%) was much higher than that in anti-PR3 group (1/16, 6.3%).
Anti-PR3 and anti-MPO occurred mainly in systemic vasculitis. A large divergence was seen in the disease spectrum between patients with anti-PR3 and those with anti-MPO. In particular, upper respiratory tract, eye and joint involvements, granuloma formation and relapse were more prominent in anti-PR3 patients. By contrast, the anti-MPO patients had a more acute disease onset, more rapid progressive renal involvement and a higher acute mortality rate.
比较抗蛋白酶3（抗PR3）或抗髓过氧化物酶（抗MPO）的抗中性粒细胞胞浆自身抗体（ANCA）患者的临床和病理表现。
采用酶联免疫吸附测定法（ELISA）对140例ANCA患者检测抗PR3和抗MPO。分析所有抗PR3或抗MPO检测呈阳性患者的就诊时临床特征、组织病理学特征及转归。
抗PR3组（n = 21）中，16例（76.2%）有系统性血管炎，其中韦格纳肉芽肿占优势（13例，61.9%）。抗MPO组（n = 31）中，19例（61.3%）被诊断为系统性血管炎，12例（38.7%）为显微镜下血管炎。对于抗PR3和抗MPO的血管炎患者，诊断时病程分别为9.6±2.0个月和4.4±0.9个月，P<0.05；血管炎活动指数（BVAS）和受累器官平均数分别为22.5±2.1、5.0±0.4和25.1±1.7、4.8±0.4，P>0.05；上呼吸道、眼和关节受累分别为11例（68.8%）、7例（43.8%）、11例（68.8%）和7例（36.8%）、2例（10.5%）、5例（26.3%），P<0.05。虽然两组间肾脏受累情况无统计学差异，但血清肌酐>500μmol/L的患者在抗MPO组比抗PR3组更常见，分别为8例（42.1%）和2例（12.5%），P<0.05。抗PR3组出现10次复发，抗MPO组仅2次，但抗MPO组的急性死亡率（5/19，27.4%）远高于抗PR3组（1/16，6.3%）。
抗PR3和抗MPO主要发生于系统性血管炎。抗PR3患者和抗MPO患者在疾病谱方面存在很大差异。特别是，抗PR3患者的上呼吸道、眼和关节受累、肉芽肿形成及复发更为突出。相比之下，抗MPO患者疾病起病更急，肾脏受累进展更快，急性死亡率更高。
