抗中性粒细胞胞浆自身抗体阴性寡免疫性肾小球肾炎的临床表现及预后
Clinical Presentation and Outcomes of Antineutrophil Cytoplasmic Autoantibody-Negative Pauci-Immune Glomerulonephritis.
作者信息
Floyd Lauren, Shetty Anamay, Morris Adam D, Galešić Krešimir, Elsayed Mohamed, Lavery Grace, Dhutia Amrita, O'Brien Sorcha, Stoneman Sinead, Egan Allyson, Little Mark A, Kratky Vojtech, Hruskova Zdenka, Tesar Vladimir, Kollar Marek, von Bergwelt-Baildon Anke, Schönermarck Ulf, Wu Eveline Y, Blazek Lauren, Derebail Vimal K, Al-Attar Mariam, Brown Nina, Álamo Beatriz Sánchez, Leung Wing-Yin, Chang Bryan, Urban Maria Letizia, Alberici Federico, Quintana Luis F, Flossmann Oliver, Brix Silke R, Geetha Duvuru, McAdoo Stephen, Dhaygude Ajay P, Kronbichler Andreas, Crnogorac Matija
机构信息
Division of Cardiovascular Sciences, The University of Manchester, Manchester, UK.
Renal Department, Royal Preston Hospital, Lancashire Teaching Hospitals, UK.
出版信息
Kidney Int Rep. 2025 Mar 3;10(5):1450-1459. doi: 10.1016/j.ekir.2025.02.032. eCollection 2025 May.
INTRODUCTION
Antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis is a rare, complex autoimmune condition. Although ANCAs have a pathogenic role, they are considered a suboptimal biomarker of disease activity. Previous studies suggest differences in clinical phenotypes and outcomes in those without detectable circulating autoantibody. This study aimed to investigate the clinical presentation, histopathological findings, treatment practices, and outcomes of patients with ANCA-negative pauci-immune glomerulonephritis (PIGN).
METHODS
A retrospective, multicenter cohort study was conducted from 2002 to 2022 and included those with biopsy-proven PIGN. We aimed to investigate differences in presentation, clinical outcomes, and treatment practices of patients with ANCA-negative PIGN when compared with ANCA-positive controls.
RESULTS
In total, 132 ANCA-negative and 127 ANCA-positive patients were included. ANCA-negative patients were younger ( < 0.001), more commonly presented with renal-limited disease ( < 0.001), had worse estimated glomerular filtration rate at diagnosis ( < 0.02) and higher rates of proteinuria ( < 0.01). Controlling for age, sex, ethnicity, and recruiting center, ANCA-negative patients had lower rates of relapse ( < 0.001) and higher rates of end-stage kidney disease (ESKD) at 1 and 3 years ( < 0.001). Standard remission induction and maintenance therapies were used less often in ANCA-negative patients.
CONCLUSION
The precise pathophysiology and factors contributing to the clinical phenotype of ANCA-negative PIGN remain unclear and potentially represent a distinct disease entity. Adverse outcomes may result from delays in diagnosis, advanced disease at presentation, and less intense immunosuppressive treatment. Current classification criteria inadequately address ANCA-negative disease and collaborative research, which includes ANCA-negative patients in trials is needed.
引言
抗中性粒细胞胞浆自身抗体（ANCA）相关血管炎是一种罕见的复杂自身免疫性疾病。尽管ANCA具有致病作用，但它们被认为是疾病活动的次优生物标志物。先前的研究表明，在那些未检测到循环自身抗体的患者中，临床表型和预后存在差异。本研究旨在调查ANCA阴性寡免疫性肾小球肾炎（PIGN）患者的临床表现、组织病理学发现、治疗方法及预后。
方法
进行了一项回顾性多中心队列研究，研究时间为2002年至2022年，纳入经活检证实为PIGN的患者。我们旨在研究ANCA阴性PIGN患者与ANCA阳性对照患者在临床表现、临床结局和治疗方法上的差异。
结果
共纳入132例ANCA阴性患者和127例ANCA阳性患者。ANCA阴性患者更年轻（<0.001），更常表现为肾脏局限性疾病（<0.001），诊断时估计肾小球滤过率更差（<0.02），蛋白尿发生率更高（<0.01）。在控制年龄、性别、种族和招募中心后，ANCA阴性患者复发率较低（<0.001），1年和3年时终末期肾病（ESKD）发生率较高（<0.001）。ANCA阴性患者较少使用标准的缓解诱导和维持治疗。
结论
ANCA阴性PIGN临床表型的确切病理生理学和影响因素仍不清楚，可能代表一种独特的疾病实体。不良结局可能源于诊断延迟、就诊时疾病进展以及免疫抑制治疗强度较低。当前的分类标准未能充分涵盖ANCA阴性疾病，需要开展包括ANCA阴性患者的协作研究。
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