Meram Medical Faculty, Department of Internal Medicine, Division of Nephrology, Necmettin Erbakan University, Konya, Turkey.
Department of Nephrology, Charles University, Faculty of Medicine and General University Hospital, Prague, Czech Republic.
Int Urol Nephrol. 2023 Nov;55(11):2817-2827. doi: 10.1007/s11255-023-03565-6. Epub 2023 Apr 3.
Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides are a group of diseases characterised by necrotizing inflammation of small vessels such as arterioles, venules, and capillaries. ANCA-associated vasculitides (AAV) are referred to as small vessel vasculitides. Three AAV subgroups, namely granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic GPA (EGPA), are defined according to clinical features. The most common disease with renal involvement in AAV is MPA Approximately 90% of patients with MPA have renal involvement. While this rate is 70-80% in GPA, less than half of EGPA patients have renal involvement. Untreated survival in AAVs is less than one year. With appropriate immunosuppressive therapy, the 5-year renal survival rate is 70-75%. Without therapy, the prognosis is poor but treatments, typically immunosuppressants, have improved survival, albeit with considerable morbidity from glucocorticoids and other immunosuppressive medications. Current challenges include improving the measures of disease activity and risk of relapse, uncertainty about optimal therapy duration and a need for targeted therapies with fewer adverse effects. In this review, we described the treatment of renal involvement in AAV in line with current studies.
抗中性粒细胞胞质抗体（ANCA）相关性血管炎是一组以小血管（如小动脉、小静脉和毛细血管）坏死性炎症为特征的疾病。ANCA 相关性血管炎（AAV）被称为小血管血管炎。根据临床特征，将三种 AAV 亚组，即肉芽肿性多血管炎（GPA）、显微镜下多血管炎（MPA）和嗜酸性粒细胞性 GPA（EGPA）进行了定义。在 AAV 中，最常见的伴有肾脏受累的疾病是 MPA。大约 90%的 MPA 患者有肾脏受累。而 GPA 中这一比例为 70-80%，不到一半的 EGPA 患者有肾脏受累。未经治疗的 AAV 患者的生存时间不到一年。接受适当的免疫抑制治疗后，5 年的肾脏存活率为 70-75%。如果不治疗，预后较差，但治疗方法，尤其是免疫抑制剂，已经改善了生存情况，尽管糖皮质激素和其他免疫抑制剂的不良反应较多。目前的挑战包括改善疾病活动度和复发风险的衡量标准、对最佳治疗持续时间的不确定性，以及对不良反应较少的靶向治疗的需求。在这篇综述中，我们根据目前的研究描述了 AAV 肾脏受累的治疗方法。
