Department of Rheumatology and Immunology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, No. 197 Ruijin Second Road, Huangpu District, Shanghai, 200025, China.
Clin Rheumatol. 2022 Nov;41(11):3439-3449. doi: 10.1007/s10067-022-06313-w. Epub 2022 Jul 29.
Patients with infective endocarditis (IE) may present rheumatic manifestations concurrent with various autoantibodies and thus mimic antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). This study aims to characterize the specific features in a long-term cohort of ANCA-positive IE patients and to perform comparative analysis with primary AAV patients.
We performed a retrospective thorough review of 475 consecutive IE patients over 23 years, identifying 22 patients positive for proteinase 3 and/or myeloperoxidase and 36 treatment-naïve AAV patients. The clinical, laboratory, and follow-up data were collected to perform comparative analysis.
Our study illustrated that ANCA-positive IE patients were younger and had a shorter duration than AAV patients. Pulmonary lesions, ENT signs, peripheral neuropath, and proteinuria were more commonly seen in AAV patients, while heart valve involvement, spleen enlargement, and cerebral hemorrhage were more typical for IE patients (all p < 0.05). Besides, ANCA-positive IE patients presented a higher level of PR3-ANCA but lower C3 (both p < 0.05). Hyperleukocytosis and thrombocytopenia were more frequently found in AAV patients (both p < 0.05). No significant difference was noticed in the survival rate.
Our study urges the early differential diagnosis of IE in ANCA-positive patients. It supports the claim that ANCA-positive IE patients and AAV patients do not share the same clinical spectrum. Echocardiography, serological profiles, and evaluation of multi-organ involvement might be required to improve diagnostic accuracy. Key Points •Early differential diagnosis of ANCA-positive IE from AAV is challenging even for expert rheumatologists. •Our study is so far one of the largest to include 22 ANCA-positive IE patients in one single center and spanning over 23 years. It is also the first study to include both ANCA-positive IE patients and AAV patients in one center. •Our study aides to identify a clinical picture to differentiate ANCA-Positive IE Patients from AAV Patients.
感染性心内膜炎（IE）患者可能同时出现风湿表现和各种自身抗体，从而模仿抗中性粒细胞胞质抗体（ANCA）相关性血管炎（AAV）。本研究旨在描述长期 ANCA 阳性 IE 患者的特定特征，并与原发性 AAV 患者进行对比分析。
我们对 23 年来的 475 例连续 IE 患者进行了回顾性全面审查，确定了 22 例蛋白酶 3 和/或髓过氧化物酶阳性和 36 例未经治疗的原发性 AAV 患者。收集了临床、实验室和随访数据进行对比分析。
我们的研究表明，ANCA 阳性 IE 患者比 AAV 患者年轻，病程更短。AAV 患者更常出现肺部病变、耳鼻喉征象、周围神经病和蛋白尿，而 IE 患者更典型的表现为心脏瓣膜受累、脾肿大和脑出血（均 p<0.05）。此外，ANCA 阳性 IE 患者的 PR3-ANCA 水平更高，但 C3 水平更低（均 p<0.05）。AAV 患者更常出现白细胞增多和血小板减少（均 p<0.05）。两组患者的生存率无显著差异。
我们的研究敦促对 ANCA 阳性患者的 IE 进行早期鉴别诊断。这支持了 ANCA 阳性 IE 患者和 AAV 患者并不具有相同临床特征的观点。需要进行超声心动图、血清学特征和多器官受累评估以提高诊断准确性。
即使是专家级别的风湿病医生，对 ANCA 阳性 IE 与 AAV 进行早期鉴别诊断也具有挑战性。
本研究是目前为止在一个中心纳入 22 例 ANCA 阳性 IE 患者并跨越 23 年的最大研究之一，也是首次在一个中心同时纳入 ANCA 阳性 IE 患者和 AAV 患者的研究。
本研究有助于确定一种临床特征，以区分 ANCA 阳性 IE 患者和 AAV 患者。
