Division of Nephrology, Columbia University College of Physicians and Surgeons, New York, New York.
Clin J Am Soc Nephrol. 2014 Mar;9(3):617-25. doi: 10.2215/CJN.07260713. Epub 2013 Sep 26.
The last decade has seen major progress in understanding the pathogenesis as well as the prognosis and treatment of patients with IgA nephropathy (IgAN). Although the diagnostic criterion of a kidney biopsy demonstrating dominant or codominant IgA deposition remains unchanged, much more is known about the genetic and environmental factors predisposing to disease development and progression. These advances have led to the identification of novel diagnostic and prognostic markers. Among the most promising clinically are genetic profiling, quantification of galactose-deficient IgA1 levels, and measurement of anti-IgA1 immunoglobulins. While targeted treatment for IgAN remains elusive, there is mounting evidence for therapeutic interventions that alter the disease course. The appropriate validation and integration of these discoveries into clinical care represent a major challenge, but one that holds tremendous promise for refining prognostication, guiding therapy, and improving the lives of patients with IgAN.
过去十年,人们对 IgA 肾病(IgAN)的发病机制、预后和治疗有了更深入的了解。虽然肾脏活检显示主要或共同占优势的 IgA 沉积的诊断标准保持不变,但人们对导致疾病发展和进展的遗传和环境因素有了更多的认识。这些进展导致了新的诊断和预后标志物的出现。其中最有前途的临床标志物包括基因谱分析、半乳糖缺乏 IgA1 水平的定量检测和抗 IgA1 免疫球蛋白的测量。虽然针对 IgAN 的靶向治疗仍难以实现,但越来越多的证据表明可以通过治疗干预来改变疾病进程。适当验证和整合这些发现以应用于临床护理是一项重大挑战,但这也为改善 IgAN 患者的预后、指导治疗和提高生活质量带来了巨大的希望。