Casal Moura Marta, Falde Sam, Sethi Sanjeev, Fervenza Fernando C, Specks Ulrich, Baqir Misbah
Division of Pulmonary and Critical Care, Department of Medicine, Mayo Clinic, Rochester, MN, USA.
Division of Nephrology and Hypertension, Department of Medicine, Mayo Clinic, Rochester, MN, USA.
Rheumatology (Oxford). 2024 Dec 1;63(12):3279-3288. doi: 10.1093/rheumatology/keae379.
ANCA-negative granulomatosis with polyangiitis (GPA) remains a diagnosis of exclusion. Clinical differences between patients with ANCA-negative vs ANCA-positive GPA have not been analysed in sizable case-control studies, and the effects of ANCA-seroconversion from negative to positive are not well documented.
A single-centre, sex and age matched case-control study evaluated ANCA-negative vs ANCA-positive GPA from 1 January 1996 to 31 December 2015. Patients who experienced seroconversion were the subject of a case-crossover study. Clinical data and outcomes were retrieved from electronic medical records.
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.
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.
抗中性粒细胞胞浆抗体（ANCA）阴性的肉芽肿性多血管炎（GPA）仍需排除其他诊断后才能确诊。在大规模病例对照研究中，尚未分析ANCA阴性与ANCA阳性GPA患者之间的临床差异，且ANCA血清学从阴性转为阳性的影响也未得到充分记录。
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患者。经历血清学转换的患者是病例交叉研究的对象。临床数据和结局从电子病历中获取。
共识别出110例ANCA阴性的GPA患者；65%为女性；诊断时的中位年龄为55岁（四分位间距39 - 65岁）。ANCA阴性的GPA患者疾病严重程度较轻（伯明翰血管炎活动评分/韦格纳肉芽肿评分[BVAS/WG]=2 vs 6，P<0.001）。黏膜/眼部表现在ANCA阴性的GPA患者中更常见。全身症状、肺部和肾脏受累在ANCA阳性的GPA患者中更常见。与ANCA阴性的GPA患者相比，ANCA阳性的GPA患者在60个月内复发更多（21.8% vs. 9.1%，P = 0.009），且事件发生时间更短（P = 0.043）。有全身表现、体重指数>30kg/m²和坏死性肉芽肿性炎症的患者更容易复发。在随访期间血清学转换为ANCA阳性的16例患者在诊断时的平均BVAS/WG更高（P<0.001），血清学转换后复发率增加（P = 0.004）。ANCA阴性的GPA患者活检时的坏死性肉芽肿性炎症被确定为随后血清学转换为ANCA阳性的危险因素。
与ANCA阳性的GPA患者相比，ANCA阴性的GPA患者疾病较轻，复发频率较低。ANCA出现预示着疾病严重程度更高和复发频率增加。
