Background ANCA-negative GPA remains a diagnosis of exclusion. Clinical differences between patients with ANCA-negative vs ANCA-positive GPA have not been analyzed in sizable case-control studies, and the effects of ANCA-seroconversion from negative to positive are not well documented. Methods A single-center, sex, and age matched case-control study evaluated ANCA-negative vs ANCA-positive GPA from January 1, 1996, to December 31, 2015. Patients who experienced seroconversion were the subject of a case crossover study. Clinical data and outcomes were retrieved from electronic medical records. Results ANCA-negative GPA was identified in 110 patients; 65% were female; median age was 55 (IQR 39–65) years at time of diagnosis. Disease severity was milder in ANCA-negative GPA (BVAS/WG = 2 vs 6, p< 0.001). Mucous membranous/eye manifestations were more frequent in ANCA-negative GPA. General symptoms, pulmonary, and renal involvement were more frequent in ANCA-positive GPA. Patients with ANCA-positive GPA relapsed more over 60 months (21.8% vs.9.1%, p= 0.009) compared with ANCA-negative GPA and had shorter time to event (p= 0.043). Patients with general manifestations, BMI > 30kg/m2 and necrotizing granulomatous inflammation were more likely to relapse. The 16 patients who seroconverted into ANCA-positive during follow-up had higher mean BVAS/WG at time of diagnosis (p< 0.001) and increased incidence of relapses (p= 0.004) after seroconversion. Necrotizing granulomatous inflammation on biopsy in ANCA-negative GPA patients was identified as a risk factor for subsequent seroconversion to ANCA-positivity. Conclusion Patients with ANCA-negative GPA have milder disease and a lower frequency of relapse than those with ANCA-positive GPA. ANCA appearance portended higher disease severity and an increased frequency of relapses.

中文翻译：

---

有或没有抗中性粒细胞胞质抗体的肉芽肿性多血管炎：病例对照研究


背景 ANCA 阴性 GPA 仍然是排除诊断。尚未在大规模病例对照研究中分析 ANCA 阴性与 ANCA 阳性 GPA 患者之间的临床差异，并且 ANCA 血清从阴性转为阳性的影响也没有得到充分记录。方法 一项单中心、性别和年龄匹配的病例对照研究评估了 1996 年 1 月 1 日至 2015 年 12 月 31 日期间 ANCA 阴性与 ANCA 阳性的 GPA。经历血清转化的患者是病例交叉研究的对象。从电子病历中检索临床数据和结果。结果 110 名患者中发现 ANCA 阴性 GPA； 65%为女性；诊断时的中位年龄为 55 (IQR 39-65) 岁。 ANCA 阴性 GPA 的疾病严重程度较轻（BVAS/WG = 2 vs 6，p< 0.001）。 ANCA 阴性 GPA 中粘膜/眼部表现更为常见。 ANCA 阳性 GPA 患者的一般症状、肺部和肾脏受累更为常见。与 ANCA 阴性 GPA 相比，ANCA 阳性 GPA 患者的复发时间超过 60 个月（21.8% vs. 9.1%，p= 0.009），并且事件发生时间更短（p= 0.043）。具有一般表现、BMI> 30kg/m2、有坏死性肉芽肿性炎症的患者复发的可能性更大。随访期间血清转化为 ANCA 阳性的 16 名患者在诊断时具有较高的平均 BVAS/WG (p< 0.001)，并且血清转化后复发率增加 (p= 0.004)。 ANCA 阴性 GPA 患者活检时出现坏死性肉芽肿性炎症，被确定为随后血清转化为 ANCA 阳性的危险因素。结论 ANCA阴性GPA患者较ANCA阳性GPA患者病情较轻，复发率较低。 ANCA 的出现预示着更高的疾病严重程度和更高的复发频率。