Division of Nephrology, Department of Internal Medicine, University of Alabama at Birmingham, Birmingham, AL, USA.
Am J Med Sci. 2021 Feb;361(2):176-194. doi: 10.1016/j.amjms.2020.10.003. Epub 2020 Oct 8.
Immunoglobulin A nephropathy (IgAN) is the most common primary glomerulonephritis worldwide. It is a leading cause of chronic kidney disease and progresses to end-stage kidney disease in up to 40% of patients about 20 years after diagnosis. Additionally, IgAN is associated with significant mortality. The diagnosis currently necessitates a kidney biopsy, as no biomarker sufficiently specific and sensitive is available to supplant the procedure. Patients display significant heterogeneity in the epidemiology, clinical manifestations, renal progression, and long-term outcomes across diverse racial and ethnic populations. Recent advances in understanding the underlying pathophysiology of the disease have led to the proposal of a four-hit hypothesis supporting an autoimmune process. To date, there is no disease-specific treatment but, with a better understanding of the disease pathogenesis, new therapeutic approaches are currently being tested in clinical trials. In this review, we examine the multiple facets and most recent advances of this interesting disease.
免疫球蛋白 A 肾病（IgAN）是全球最常见的原发性肾小球肾炎。它是慢性肾脏病的主要病因之一，约有 40%的患者在诊断后 20 年内进展为终末期肾病。此外，IgAN 与显著的死亡率相关。目前，该疾病的诊断需要进行肾活检，因为没有足够特异和敏感的生物标志物来替代该程序。在不同的种族和民族人群中，患者在流行病学、临床表现、肾脏进展和长期结局方面表现出显著的异质性。对疾病潜在病理生理学的深入理解最近取得了进展，提出了一个支持自身免疫过程的四击假说。迄今为止，还没有针对该疾病的特异性治疗方法，但随着对疾病发病机制的深入了解，新的治疗方法目前正在临床试验中进行测试。在这篇综述中，我们探讨了这种有趣疾病的多个方面和最新进展。
