背景:ANCA阴性GPA仍然是一种排除性诊断。在相当大的病例对照研究中,尚未分析ANCA阴性与ANCA阳性GPA患者之间的临床差异。ANCA-血清从阴性到阳性的转换效果没有很好的记录。
方法:单中心,性别,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ANCA阴性与ANCA阳性GPA.经历血清转换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:在110例患者中发现ANCA阴性GPA;65%为女性;诊断时的中位年龄为55(IQR39-65)岁。ANCA阴性GPA的疾病严重程度较轻(BVAS/WG=2vs6,p<0.001)。在ANCA阴性GPA中,粘膜/眼表现更为常见。一般症状,肺,ANCA阳性GPA的肾脏受累频率更高。ANCA阳性GPA患者在60个月内复发更多(21.8%vs.9.1%,p=0.009)与ANCA阴性GPA相比,事件发生时间更短(p=0.043)。有一般表现的患者,BMI>30kg/m2和坏死性肉芽肿性炎症更容易复发。在随访期间血清转化为ANCA阳性的16例患者在诊断时具有较高的平均BVAS/WG(p<0.001),血清转化后复发的发生率增加(p=0.004)。在ANCA阴性GPA患者的活检中,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患者的病情较轻,复发频率低于ANCA阳性GPA患者。ANCA的出现预示着更高的疾病严重程度和复发频率的增加。
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.
CONCLUSIONS: 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.