Department of Pediatrics, Peking University First Hospital, Beijing, China.
Pediatr Nephrol. 2023 Jun;38(6):1707-1715. doi: 10.1007/s00467-022-05798-6. Epub 2022 Nov 8.
IgA nephropathy (IgAN) and IgA vasculitis-associated nephritis (IgAVN) are among the most frequent childhood glomerular diseases and are characterized by significant variability in clinical manifestations, pathological presentation and long-term outcomes. IgAVN, alternatively called purpura nephritis, is pathologically indistinguishable from kidney-limited IgAN. In Chinese children, the clinical presentations and pathological manifestations of IgAN and IgAVN are variable. The severity of proteinuria and abnormalities in kidney function and blood pressure of children in China are comparable to those of children in Europe, the USA, and Japan. Compared to Caucasian children and Japanese children, crescents were more common in Chinese children with IgAN or IgAVN. Approximately 10-20% of childhood IgAN or IgAVN progresses to impaired kidney function in China. Since 2007, a series of guidelines on the diagnosis and treatment of pediatric kidney diseases has been published following the principles of evidence-based medicine. However, a large difference exists between the Chinese evidence-based guidelines and the guidelines developed by Kidney Disease: Improving Global Outcomes (KDIGO) in 2021. Chinese children with IgAN or IgAVN were more likely to be treated with steroids or immunosuppressive agents. Further studies exploring the optimal treatment regimen for childhood IgAN or IgAVN are needed in the future.
IgA 肾病 (IgAN) 和 IgA 血管炎相关性肾炎 (IgAVN) 是儿童中最常见的肾小球疾病之一,其临床表现、病理表现和长期预后存在显著差异。IgAVN,又称紫癜性肾炎,在病理上与局限于肾脏的 IgAN 无法区分。在中国儿童中,IgAN 和 IgAVN 的临床表现和病理表现各不相同。中国儿童蛋白尿、肾功能和血压异常的严重程度与欧美日儿童相当。与高加索儿童和日本儿童相比,IgAN 或 IgAVN 患儿的新月体更为常见。在中国,约 10-20%的儿童 IgAN 或 IgAVN 进展为肾功能受损。自 2007 年以来,根据循证医学原则发布了一系列关于儿科肾脏疾病的诊断和治疗指南。然而,中国的循证指南与 2021 年肾脏病:改善全球预后组织 (KDIGO) 制定的指南之间存在很大差异。中国 IgAN 或 IgAVN 患儿更倾向于接受类固醇或免疫抑制剂治疗。未来需要进一步研究探索儿童 IgAN 或 IgAVN 的最佳治疗方案。