Division of Nephrology and Rheumatology, RWTH Aachen University Hospital, Pauwelsstr. 30, 52074, Aachen, Germany.
Division of Nephrology, Department of Medicine, The University of Hong Kong, Queen Mary Hospital, Pok Fu Lam, Hong Kong.
Semin Immunopathol. 2021 Oct;43(5):717-728. doi: 10.1007/s00281-021-00888-3. Epub 2021 Sep 8.
IgA nephropathy (IgAN) is the most common type of glomerulonephritis in Asia and the Western world. In most patients, it follows an asymptomatic to oligosymptomatic course and GFR loss, if any, is slow. The mainstay of therapy therefore is optimized supportive care, i.e., measures that lower blood pressure, reduce proteinuria, minimize lifestyle risk factors, and otherwise help to reduce non-specific insults to the kidneys. The value of immunosuppression has become controversial and if at all, systemic high-dose corticosteroid therapy should be considered for a few months taking into account patient characteristics that would caution against or preclude such therapy. In addition, adverse events related to corticosteroid therapy markedly increase as GFR declines. Beyond corticosteroids, there is little evidence that any additional immunosuppression is helpful, with the exception of mycophenolate mofetil in patients of Asian descent. A considerable number of clinical trials ranging from enteric coated budesonide to blockade of B-cell function to complement inhibitors are currently ongoing and will hopefully allow a more targeted therapy of high-risk patients with progressive IgAN in the future.
IgA 肾病(IgAN)是亚洲和西方世界最常见的肾小球肾炎类型。在大多数患者中,它表现为无症状至少症状性,且肾小球滤过率(GFR)损失缓慢。因此,治疗的主要方法是优化支持性治疗,即降低血压、减少蛋白尿、最小化生活方式风险因素等措施,以减少对肾脏的非特异性损伤。免疫抑制的价值变得有争议,如果有,应考虑对有特定特征的患者进行数月的全身性大剂量皮质类固醇治疗,这些特征提示或排除这种治疗。此外,随着 GFR 下降,皮质类固醇治疗相关的不良反应显著增加。除了皮质类固醇,几乎没有证据表明任何额外的免疫抑制治疗是有益的,除了亚洲裔患者使用霉酚酸酯。目前正在进行大量的临床试验,范围从肠溶性布地奈德到 B 细胞功能阻断到补体抑制剂,有望在未来为进展性 IgAN 的高危患者提供更有针对性的治疗。
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