Zhang Yuxin, Xu Gaosi
Department of Nephrology, the Second Affiliated Hospital, Jiangxi Medical College, Nanchang University, Nanchang, Jiangxi; and Grade 2021, the Second Clinical Medical College of Nanchang University, Nanchang, Jiangxi, China.
Department of Nephrology, the Second Affiliated Hospital, Jiangxi Medical College, Nanchang University, Nanchang, Jiangxi, China;.
Clin Exp Rheumatol. 2025 Apr;43(4):728-741. doi: 10.55563/clinexprheumatol/mjhyff. Epub 2024 Oct 8.
IgA vasculitis with nephritis (IgAVN) is closely related to IgA nephritis (IgAN) and IgA vasculitis (IgAV), but the clinical characteristics and exact pathogenesis of IgAVN remain unclear. In the present study, we have reviewed 8 clinical trials with different treatments and found that most IgAVN patients had partial recovery after treatments while few patients (26.5%) recovered completely within 6 months. Adding cyclophosphamide to mycophenolate mofetil was beneficial in children with severe kidney damage but was not effective in adults with serious organ damage (p=0.847). Tonsillectomy reduced the recurrence rate (p=0.03). In 18 reported cases we summarised, intravenous methylprednisolone pulse (MEP) combined with immunosuppressants (66.7%) and MEP combined with oral prednisolone (27.8%) were the two most commonly utilised treatments, and rituximab (40%) was the most frequently used monoclonal antibody. Mechanistically, activated cytotoxic T lymphocytes, natural killer cells, macrophage and completements contributed to the inflammation and endothelial cell apoptosis in IgAVN patients. Galactose-deficient IgA1 may be a threshold for IgAVN. The bulk formation of immune complexes and the decreased clearance rate led to the deposition of immune complexes. In severe cases, coagulation cascade would be triggered and thus caused renal fibrosis.
IgA 血管炎伴肾炎（IgAVN）与 IgA 肾病（IgAN）和 IgA 血管炎（IgAV）密切相关，但 IgAVN 的临床特征和确切发病机制仍不清楚。在本研究中，我们回顾了 8 项不同治疗方法的临床试验，发现大多数 IgAVN 患者治疗后有部分恢复，而少数患者（26.5%）在 6 个月内完全恢复。在霉酚酸酯中添加环磷酰胺对严重肾损伤儿童有益，但对有严重器官损伤的成人无效（p = 0.847）。扁桃体切除术降低了复发率（p = 0.03）。在我们总结的 18 例报告病例中，静脉注射甲泼尼龙冲击（MEP）联合免疫抑制剂（66.7%）和 MEP 联合口服泼尼松龙（27.8%）是最常用的两种治疗方法，利妥昔单抗（40%）是最常用的单克隆抗体。从机制上讲，活化的细胞毒性 T 淋巴细胞、自然杀伤细胞、巨噬细胞和补体导致 IgAVN 患者的炎症和内皮细胞凋亡。缺乏半乳糖的 IgA1 可能是 IgAVN 的一个阈值。免疫复合物的大量形成和清除率降低导致免疫复合物沉积。在严重情况下，会触发凝血级联反应，从而导致肾纤维化。
