感染性心内膜炎(IE)可误诊为ANCA相关性血管炎(AAV),特别是当检测到抗中性粒细胞胞浆抗体(ANCA)时。区分IE和AAV对于指导治疗至关重要。然而,对IE患者的ANCA阳性知之甚少。我们提供了关于ANCA阳性IE患者的病例报告和文献的系统综述,旨在提供该实体的全面概述,并在遇到类似病例时帮助临床医生做出决定。根据PRISMA-IPD指南,在PubMed上对先前没有AAV诊断的ANCA阳性IE原始病例的论文进行了系统审查。一组预先定义的临床,实验室,我们提取了每位患者的肾活检结果,并以叙事和定量综合的形式呈现.共纳入74份报告,描述了181例ANCA阳性IE患者(共182例,包括我们自己的病例)。在18-43%的IE患者中发现ANCA阳性。患者通常表现为亚急性IE(73%),细胞质ANCA染色或抗蛋白酶3抗体阳性(79%)。72%的肾功能受损;肾脏活检结果提示59%的免疫复合物,而37%的人表现出浅层免疫性肾小球肾炎。所有患者均接受抗生素治疗;39%的患者还接受了免疫抑制剂治疗。随访期间,69%的患者为ANCA阴性,未报告诊断为系统性血管炎。这项研究回顾了迄今为止最大的ANCA阳性IE患者系列,并显示了IE和AAV之间的临床表现重叠。因此,我们强调,临床医生在治疗疑似AAV患者时,应警惕潜在感染的可能性。即使被ANCA的积极性所保证。本系统综述描述了迄今为止最大的ANCA阳性感染性心内膜炎(IE)患者系列(N=182),并显示IE和ANCA相关性血管炎(AAV)之间的临床表现高度重叠。•在18-43%的感染性心内膜炎患者中发现ANCA阳性。在ANCA阳性IE患者中,大多数(79%)显示细胞质ANCA染色或抗PR3抗体.我们强调,临床医生在治疗疑似AAV患者时,应警惕潜在感染的可能性。即使被ANCA的积极性所保证。•在患有IE和ANCA相关症状如急性肾损伤的患者中,一个重要的临床挑战是开始免疫抑制治疗。所有具有此系列数据的患者均接受抗生素治疗;39%还接受了免疫抑制治疗。在许多这样的患者中,感染治疗后,ANCA相关症状缓解或稳定。ANCA滴度在69%变为阴性,所有病例均未诊断为AAV。因此,我们建议(经验性)抗生素治疗仍然是ANCA阳性IE患者的治疗基石。同时建议对免疫抑制采取谨慎的观望态度。
Infective endocarditis (IE) may be misdiagnosed as ANCA-associated vasculitis (AAV), especially when antineutrophil cytoplasmic antibodies (ANCA) are detected. Distinguishing IE from AAV is crucial to guide therapy. However, little is known about ANCA positivity in IE patients. We present a
case report and systematic review of the literature on patients with ANCA-positive IE, aiming to provide a comprehensive overview of this entity and to aid clinicians in their decisions when encountering a similar
case. A systematic review of papers on original cases of ANCA-positive IE without a previous diagnosis of AAV was conducted on PubMed in accordance with PRISMA-IPD guidelines. A predefined set of clinical, laboratory, and kidney biopsy findings was extracted for each patient and presented as a narrative and quantitative synthesis. A total of 74
reports describing 181 patients with ANCA-positive IE were included (a total of 182 cases including our own
case). ANCA positivity was found in 18-43% of patients with IE. Patients usually presented with subacute IE (73%) and had positive cytoplasmic ANCA-staining or anti-proteinase-3 antibodies (79%). Kidney function was impaired in 72%; kidney biopsy findings were suggestive of immune complexes in 59%, while showing pauci-immune glomerulonephritis in 37%. All were treated with antibiotics; 39% of patients also received immunosuppressants. During follow-up, 69% of patients became ANCA-negative and no diagnosis of systemic vasculitis was reported. This study reviewed the largest series of patients with ANCA-positive IE thus far and shows the overlap in clinical manifestations between IE and AAV. We therefore emphasize that clinicians should be alert to the possibility of an underlying infection when treating a patient with suspected AAV, even when reassured by ANCA positivity. Key Points • This systematic review describes - to our knowledge - the largest series of patients with ANCA-positive infective endocarditis (IE) thus far (N=182), and shows a high degree of overlap in clinical manifestations between IE and ANCA-associated vasculitis (AAV). • ANCA positivity was found in 18-43% of patients with infective endocarditis. Of patients with ANCA-positive IE, the majority (79%) showed cytoplasmic ANCA-staining or anti-PR3-antibodies. We emphasize that clinicians should be alert to the possibility of an underlying infection when treating a patient with suspected AAV, even when reassured by ANCA positivity. • In patients with IE and ANCA-associated symptoms such as acute kidney injury, an important clinical challenge is the initiation of immunosuppressive therapy. All patients with data in this series received antibiotics; 39% also received immunosuppressive therapy. In many of these patients, ANCA-associated symptoms resolved or stabilized after infection was treated. ANCA titers became negative in 69% , and a diagnosis of AAV was made in none of the cases. We therefore recommend that (empiric) antibiotic treatment remains the therapeutic cornerstone for ANCA-positive IE patients, while a watchful wait-and-see approach with respect to immunosuppression is advised.