抗髓过氧化物酶抗体阳性的韦格纳肉芽肿病的特点

目的 分析抗髓过氧化物酶(MPO)抗体阳性的韦格纳肉芽肿病(WG)患者的临床病理特点及其与传统的抗蛋白酶3(PR3)抗体阳性者之间的差异&#65377;方法 89例WG患者经本院肾内科确诊,分析其临床病理资料并对比抗MPO抗体阳性和抗PR3抗体阳性的两组患者之间的差异&#65377;结果 89例患者中54例抗MPO抗体阳性,34例抗PR3抗体阳性&#65377;抗MPO抗体阳性患者中男性所占的比例显著低于抗PR3抗体阳性者(男:女分别为23:31与24:10, P<0.05)&#65377;抗MPO抗体阳性的WG临床亦呈多器官受累的表现,其中关节痛&#65380;皮疹&#65380;眼&#65380;耳受累的发生率显著低于抗PR3抗体阳性者(分别为46.3%比70.6%,P < 0.05; 20.4%比44.1%,P < 0.05;27.8%比58.8%,P < 0.01和40.7%比67.6%,P < 0.05);伯明翰血管炎活动度积分(BVAS)显著低于抗PR3抗体阳性者(22.2±6.2比24.7±6.9, P < 0.05);而在确诊时Scr增高的发生率则显著高于抗PR3抗体阳性者(81.5%比61.8%, P < 0.05)&#65377;结论 在国人的WG患者中,抗MPO抗体阳性者可能不占少数,它与抗PR3抗体阳性者临床表现有所不同&#65377;

Clinicopathological characteristics of Wegener granulomatosis patients with positive anti-myeloperoxidase autoantibodies

Objective To analyze the clinical and pathological characteristics of patients with MPO-ANCA positive Wegener granulomatosis(WG)and to investigate the difference between patients with MPO-ANCA and PR3-ANCA. Methods Patients with WG were selected according to both Chapel Hill Consensus Conference definition and American College of Rheumatology (ACR) classification criteria in 700 Chinese patients with ANCA-positive systemic vasculitis. The clinical manifestions were compared between patients with MPO-ANCA and with PR3-ANCA. Results Eighty-nine patients fulfilled the diagnostic criteria of WG, 54/89(60.7%) were MPO-ANCA positive, 34/89(38.2%) were PR3-ANCA positive, and the rest one was both positive. Patients with MPO-ANCA were female predominant compared with patients with PR3-ANCA, their male/female ratios were 23:31 vs 24:10, respectively(P < 0.05). Patients with MPO-ANCA also had multi-system involvement. However, the prevalences of arthagia, skin rash, ophthalmic and ear involvement were significantly lower in patients with MPO-ANCA than those in patients with PR3-ANCA (46.3% vs 70.6%, P <0.05; 20.4% vs 44.1%, P < 0.05; 27.8% vs 58.8%, P < 0.01; 40.7% vs 67.6%, P < 0.05; respectively). The prevalence of elevated initial serum creatinine was significantly higher in patients with MPO-ANCA than that in patients with PR3-ANCA (81.5% vs 61.8%, χ2=4.2, P < 0.05). The mean score of Birmingham vasculitis activity score (BVAS) was significantly lower in patients with MPO-ANCA than that in patients with PR3-ANCA(22.2±6.21 vs 24.7±6.87, P < 0.05). Conclusions Patients with MPO-ANCA positive WG were common in Chinese. The significance of patients with WG with different ANCA serotypes still needs further investigation.