Amatruda Michela, Carucci Nicolina Stefania, Chimenz Roberto, Conti Giovanni
Pediatric Nephrology and Rheumatology Unit, AOU G Martino, University of Messina, Messina 98125, Italy.
World J Nephrol. 2023 Sep 25;12(4):82-92. doi: 10.5527/wjn.v12.i4.82.
The clinical spectrum of immunoglobulin A vasculitis nephritis (IgAVN) ranges from the relatively common transitory microscopic hematuria and/or low-grade proteinuria to nephritic or nephrotic syndrome, rapidly progressive glomerulonephritis, or even renal failure. Clinical and experimental studies have shown a multifactor pathogenesis: Infection triggers, impaired glycosylation of IgA1, complement activation, Toll-like-receptor activation and B cell proliferation. This knowledge can identify IgAVN patients at a greater risk for adverse outcome and increase the evidence for treatment recommendations.
免疫球蛋白A血管炎肾病(IgAVN)的临床谱范围较广,从相对常见的短暂性镜下血尿和/或轻度蛋白尿到肾炎或肾病综合征、快速进展性肾小球肾炎,甚至肾衰竭。临床和实验研究已表明其发病机制具有多因素性:感染触发因素、IgA1糖基化受损、补体激活、Toll样受体激活和B细胞增殖。这些认识有助于识别预后不良风险较高的IgAVN患者,并为治疗建议提供更多依据。