Faculty of Medicine, Wroclaw Medical University, Wrocław, Poland.
Department of Clinical and Experimental Pathology, Wroclaw Medical University, Wrocław, Poland.
Front Immunol. 2023 Aug 15;14:1227878. doi: 10.3389/fimmu.2023.1227878. eCollection 2023.
Although associations of IgA nephropathy (IgAN) and ANCA-associated vasculitis (AAV) have been described, this coexistence scarcely occurs and requires multidisciplinary management. Herein, we discuss a course of treatment introduced in a patient with two exacerbations. Furthermore, alterations in histopathological images between two kidney biopsies are presented. The applicability of traditional inflammatory markers, e.g., CRP, in monitoring disease severity in AAV and IgAN is limited. Based on our patient and current literature, we suggest ANCA testing in patients with rapidly progressing IgAN for therapeutic and prognostic purposes. As regards the therapy of IgAN associated with AAV, aggressive immunosuppressive regimens with methylprednisolone and cyclophosphamide are recommended. Alternatively, methylprednisolone with rituximab, plasma exchange, mycophenolate mofetil, and intravenous immunoglobulin (IVIG) could also be considered.
虽然 IgA 肾病 (IgAN) 和抗中性粒细胞胞浆抗体相关性血管炎 (AAV) 之间存在关联,但这种共存情况很少见,需要多学科管理。在此,我们讨论了一位患者在两次发作中引入的治疗方案。此外,还介绍了两次肾活检之间组织病理学图像的变化。传统炎症标志物(如 CRP)在监测 AAV 和 IgAN 疾病严重程度方面的适用性有限。基于我们的患者和当前文献,我们建议对快速进展性 IgAN 患者进行 ANCA 检测,以达到治疗和预后目的。对于 IgAN 合并 AAV 的治疗,建议采用甲基强的松龙和环磷酰胺等强化免疫抑制方案。或者,可以考虑使用甲基强的松龙联合利妥昔单抗、血浆置换、霉酚酸酯和静脉注射免疫球蛋白 (IVIG)。