摘要：目的：探讨抗中性粒细胞胞浆抗体(ANCA)类型与ANCA相关性血管炎(AAV)诊断与人口学特征、疾病表现及临床结局的关系。我们关注患者ANCA类型和疾病类型分类之间差异：抗髓过氧化物酶(MPO)-ANCA阳性和ANCA阴性的肉芽肿性多血管炎(Wegener's)(GPA)患者的差异。

        方法：我们对依那西普治疗GPA患者的试验和利妥昔单抗治疗MPO-ANCA阳性的GPA和ANCA阴性的GPA患者,以及蛋白酶3(PR3)-ANCA阳性GPA和MPO-ANCA阳性显微镜下多血管炎(MPA)患者的研究进行汇总分析。

        结果：365例患者中，273例(75%)为PR3-ANCA阳性GPA，33例(9%)为MPO-ANCA阳性GPA，15例(4%)为ANCA阴性GPA，44例(12%)为MPO-ANCA阳性的MPA。与MPO-ANCA阳性MPA患者相比，MPO-ANCA阳性GPA患者的诊断年龄更小(53岁比61岁; P = 0.02)。他们的疾病表现和复发率与PR3-ANCA阳性GPA患者相似。在MPO-ANCA阳性的GPA患者中，疾病复发在入组时以及12和18个月时较MPO-ANCA阳性的MPA患者更为常见。ANCA阴性的GPA患者在试验入组时伯明翰血管炎评分低于PR3-ANCA阳性的GPA患者(4.5 vs. 7.7; P <0.01)，主要因为肾脏受累发生率较低。

        结论：我们无法证实MPO-ANCA阳性和PR3-ANCA阳性的GPA患者之间的重要临床差异。在该患者队列中，患者复发风险的大小与疾病类型相关更密切，而非ANCA类型。这些结果值得在评估AAV患者复发风险时考虑。

        附全文: Abstract OBJECTIVE: To examine the relationship of antineutrophil cytoplasmic antibody (ANCA) type and ANCA-associated vasculitis (AAV) diagnosis with demographic features, disease manifestations, and clinical outcomes. We focused on patients who account for the differences between ANCA type and disease type classifications: anti-myeloperoxidase (MPO)-ANCA-positive and ANCA-negative patients with granulomatosis with polyangiitis (Wegener's) (GPA). METHODS: We performed a pooled analysis of the Wegener's GranulomatosisEtanercept Trial and the Rituximab in ANCA-Associated Vasculitis trial comparing patients with MPO-ANCA-positive GPA and patients with ANCA-negative GPA to patients with proteinase 3 (PR3)-ANCA-positive GPA and patients with MPO-ANCA-positive microscopic polyangiitis (MPA). RESULTS: Of the 365 patients analyzed, 273 (75%) had PR3-ANCA-positive GPA, 33 (9%) had MPO-ANCA-positive GPA, 15 (4%) had ANCA-negative GPA, and 44 (12%) had MPO-ANCA-positive MPA. MPO-ANCA-positive GPA patients were younger at diagnosis compared to MPO-ANCA-positive MPA patients (53 versus 61 years; P = 0.02). Their disease manifestations and rates of relapse were similar to those of PR3-ANCA-positive GPA patients. Relapse was more frequent in MPO-ANCA-positive GPA patients than in patients with MPO-ANCA-positive MPA at trial entry as well as at 12 and 18 months. ANCA-negative patients with GPA had lower Birmingham Vasculitis Activity Score for Wegener's Granulomatosis scores at trial entry than PR3-ANCA-positive patients with GPA (4.5 versus 7.7; P < 0.01), primarily because of a lower prevalence of renal involvement. CONCLUSION: We were unable to demonstrate important clinical differences between MPO-ANCA-positive and PR3-ANCA-positive patients with GPA. The risk of relapse was associated more closely with disease type than with ANCA type in this patient cohort. These findings deserve consideration in the assessment of relapse risk in patients with AAV.

        引自: Miloslavsky EM1, Lu N1, Unizony S1, et al.Myeloperoxidase-Antineutrophil Cytoplasmic Antibody (ANCA)-Positive and ANCA-Negative Patients WithGranulomatosis With Polyangiitis (Wegener's): Distinct Patient Subsets. Arthritis Rheumatol. 2016 Dec;68(12):2945-2952. doi: 10.1002/art.39812.