Franssen C, Gans R, Kallenberg C, Hageluken C, Hoorntje S
Department of Internal Medicine, University Hospital, Groningen, The Netherlands.
J Intern Med. 1998 Sep;244(3):209-16. doi: 10.1046/j.1365-2796.1998.00357.x.
To compare the disease spectrum of consecutive patients with antineutrophil cytoplasmic autoantibodies directed against proteinase 3 (anti-PR3) or myeloperoxidase (anti-MPO).
Retrospective analysis.
Three teaching hospitals in the Netherlands.
Clinical features at presentation, histopathological characteristics and outcome.
All consecutive patients who tested positive for anti-PR3 (n=46) or anti-MPO (n=46) over an 8-year-period.
At diagnosis, patients with anti-PR3 had a higher vasculitis activity index than patients with anti-MPO (P < 0.001). The mean (SD) number of affected organs in the anti-PR3 group exceeded that of the anti-MPO group (3.9 (1.4) and 2.2 (1.1), respectively; P < 0.01). The combination of renal and respiratory tract involvement was present in as many as 78.3% of patients with anti-PR3 and in only 23.9% of patients with anti-MPO (P < 0.01). Renal-limited disease exclusively occurred in patients with anti-MPO. Granulomas were found in 41.3% of anti-PR3- but in only 4.3% of anti-MPO-positive patients (P < 0.01). All anti-PR3-positive patients had Wegener's granulomatosis or microscopic polyangiitis. By contrast, diagnoses in the anti-MPO group were more diverse: idiopathic necrotizing crescentic glomerulonephritis (26.1%), microscopic polyangiitis (26.1%). Churg-Strauss syndrome (4.3%), Wegener's granulomatosis (2.2%), giant cell arteritis (2.2%), clinically suspected vasculitis (19.6%), as well as miscellaneous nonvasculitic disorders (19.6%). During follow-up, 10 anti-PR3-positive patients had 11 relapses whereas only 3 patients with anti-MPO relapsed (P=0.04).
A large divergence was seen in the disease spectrum between patients with anti-PR3 and those with anti-MPO. In particular, extra-renal disease manifestations, granuloma formation and relapses were more prominent in anti-PR3- than in anti-MPO-positive patients.
比较连续的抗蛋白酶3抗中性粒细胞胞浆抗体（抗PR3）或抗髓过氧化物酶抗体（抗MPO）患者的疾病谱。
回顾性分析。
荷兰的三家教学医院。
就诊时的临床特征、组织病理学特征及转归。
8年间所有抗PR3（n = 46）或抗MPO（n = 46）检测呈阳性的连续患者。
诊断时，抗PR3患者的血管炎活动指数高于抗MPO患者（P < 0.001）。抗PR3组受累器官的平均（标准差）数量超过抗MPO组（分别为3.9（1.4）和2.2（1.1）；P < 0.01）。抗PR3患者中多达78.3%出现肾脏和呼吸道受累，而抗MPO患者中仅23.9%出现（P < 0.01）。仅肾脏受累的疾病仅发生在抗MPO患者中。41.3%的抗PR3阳性患者发现有肉芽肿，而抗MPO阳性患者中仅4.3%有（P < 0.01）。所有抗PR3阳性患者均患有韦格纳肉芽肿或显微镜下多血管炎。相比之下，抗MPO组的诊断更多样化：特发性坏死性新月体性肾小球肾炎（26.1%）、显微镜下多血管炎（26.1%）、变应性肉芽肿性血管炎（4.3%）、韦格纳肉芽肿（2.2%）、巨细胞动脉炎（2.2%）、临床疑似血管炎（19.6%）以及其他非血管炎性疾病（19.6%）。随访期间，10例抗PR3阳性患者复发11次，而抗MPO患者仅有3例复发（P = 0.04）。
抗PR3患者与抗MPO患者的疾病谱存在很大差异。特别是，抗PR3阳性患者的肾外疾病表现、肉芽肿形成和复发比抗MPO阳性患者更突出。
