单中心的PR3-ANCA阳性患者50例临床分析

Clinical Analysis of 50 PR3-ANCA Positive Patients in a Single Center

目的探讨PR3-ANCA阳性患者的临床特点,提高对非原发性血管炎患者的认识和警觉性,以减少误诊误治。方法筛选2017年1月1日至2022年1月1日于某院就诊的患者,筛选条件为至少有1次PR3-ANCA检测结果为阳性,收集患者的临床及实验室资料,并进行统计分析。结果按照PR3-ANCA阳性的标准,共纳入50例患者。其中仅有15例患者诊断为原发性AASV,占全部患者的30%;另外35例(70%)为非原发性AASV患者,其中主要诊断为结缔组织病(14例)、感染性疾病(6例)、恶性疾病(6例)以及其他疾病(9例)。在PR3-ANCA及C-ANCA双阳性患者中(n=8),仍有4例患者诊断为非原发性AASV疾病。按照是否为原发性AASV分组,比较了的两组患者的血PR3-ANCA的水平,发现与原发性AASV患者相比,非原发性AASV患者的血PR3-ANCA水平较低[55.4(34.2-134.6)对82.2(58.7-400),P=0.037],差异有统计学意义,但将这部分患者的主要诊断按照自身免疫病、恶性疾病及感染性疾病分亚组后,再次与原发性AASV患者进行比较,发现PR3-ANCA水平在组间并没有统计学差异(P=0.225)。再次比较PR3-ANCA阳性的原发性AASV与非原发性AASV患者的临床表现以及器官系统受累情况,发现与非原发性AASV患者相比,原发性AASV患者更易出现发热、乏力及体重减轻等非特异症状(86.7%对比35.3%,P=0.001),差异有统计学意义。结论在PR3-ANCA阳性的患者中,需考虑存在非AASV疾病的可能,以系统性红斑狼疮、干燥综合征、溃疡性结肠炎、骨髓增生异常综合征、淋巴瘤、消化系统肿瘤以及感染性心内膜炎多见。

Objectives To investigate the clinical characteristics of PR3-ANCA positive patients,and to improve the awareness and vigilance of non-primary vasculitis patients,so as to reduce misdiagnosis and mismanagement.Methods Patients admitted to a hospital from January 1st,2017 to January 1st,2022 were selected.The screening conditions were that at least one PR3-ANCA test was positive,and clinical and laboratory data of patients were collected for statistical analysis.Results A total of 50 patients were enrolled according to PR3-ANCA positive criteria.Only 15 patients were diagnosed as primary ANCA associated systemic vasculitis(AASV),accounting for 30%of all patients.Another 35 patients(70%)were non-primary AASV patients,of whom the main diagnoses were connective tissue disease(n=14),infectious disease(n=6),malignant disease(n=6),and other diseases(n=9).Among PR3-ANCA and cytoplasmic ANCA(C-ANCA)double-positive patients(n=8),4patients were still diagnosed with non-primary AASV disease.In this study,the PR3-ANCA level of patients in the two groups was compared,and it was found that the PR3-ANCA level of patients with non-primary AASV was lower than that of patients with primary AASV[55.4(34.2-134.6)vs.82.2(58.7-400),P=0.037],and the difference was statistically significant.However,these patients were grouped according to the main diagnosis of autoimmune disease,malignant disease and infectious disease,and compared with patients with primary AASV again,there was no statistical difference in PR3-ANCA level between groups(P=0.225).This study further compared the clinical manifestations and organ system involvement of patients with PR3-ANCA positive primary AASV and non-primary AASV,and found that patients with primary AASV were more likely to have non-specific symptoms such as fever,fatigue and weight loss compared with non-primary AASV patients(86.7%compared with 35.3%,P=0.001),the difference was statistically significant.Conclusions In patients with positive PR3-ANCA,the possibility of non-AASV diseases should be considered,and systemic lupus erythematosus,sjogren's syndrome,ulcerative colitis,myelodysplastic syndrome,lymphoma,digestive tumor and infective endocarditis are more common.