Wang Weiming, Chen Nan
Department of Nephrology, Ruijin Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai, PR China.
Contrib Nephrol. 2013;181:75-83. doi: 10.1159/000348460. Epub 2013 May 8.
IgA nephropathy (IgAN) is the most common primary glomerular disease worldwide. About 25-30% of IgAN patients will progress to end-stage kidney disease in 20-25 years. Early-onset symptoms that are highly suggestive of progressive IgAN include massive proteinuria, hypertension, renal damage, glomerular sclerosis, crescent formation, and tubulointerstitial fibrosis. Progressive IgAN may progress to renal failure in a short time. Optimized supportive therapy is the fundamental treatment for progressive IgAN patients, and includes renin-angiotensin system blockers, blood pressure control, antiplatelet and anticoagulant drugs, statins, and allopurinol. In progressive IgAN patients whose clinical and pathological manifestations are more severe, active therapy may be considered including glucocorticoid therapy, cyclophosphamide, azathioprine, mycophenolate mofetil, tacrolimus, and other immunosuppressants. However, there are currently controversies on the definition and treatment of progressive IgAN.
IgA肾病(IgAN)是全球最常见的原发性肾小球疾病。约25%-30%的IgAN患者将在20-25年内进展为终末期肾病。高度提示进行性IgAN的早期症状包括大量蛋白尿、高血压、肾损伤、肾小球硬化、新月体形成和肾小管间质纤维化。进行性IgAN可能在短时间内进展为肾衰竭。优化的支持性治疗是进行性IgAN患者的基本治疗方法,包括肾素-血管紧张素系统阻滞剂、血压控制、抗血小板和抗凝药物、他汀类药物和别嘌醇。对于临床和病理表现更严重的进行性IgAN患者,可考虑积极治疗,包括糖皮质激素治疗、环磷酰胺、硫唑嘌呤、霉酚酸酯、他克莫司和其他免疫抑制剂。然而,目前关于进行性IgAN的定义和治疗存在争议。