Division of Nephrology and Hypertension, Saint George Hospital University Medical Center, PO Box 166 378 Achrafieh, Beirut 11 00 2807, Lebanon. Electronic address: https://twitter.com/sabinekaram6.
Division of Nephrology and Hypertension, Division of Hematology, Mayo Clinic, 200 1st Street Southwest, Rochester, MN 55905, USA.
Hematol Oncol Clin North Am. 2020 Dec;34(6):1069-1079. doi: 10.1016/j.hoc.2020.08.002. Epub 2020 Sep 26.
Kidney involvement in immunoglobulin-related amyloidosis (AIg) is common. Although patients with renal-limited AIg tend not to have the high mortality that patients with cardiac amyloidosis have, they do experience significant morbidity and impact on quality of life. The complexity of the pathogenesis remains incompletely understood. Models have been established to prognosticate and assess for the response to therapy. Patients with advanced renal impairment from immunoglobulin light chain amyloidosis still have poor renal prognosis, and better therapy is needed in order to preserve kidney function. Patients who develop end-stage renal disease can undergo renal replacement therapy with kidney transplantation.
肾脏受累于免疫球蛋白相关淀粉样变性（AIg）较为常见。虽然肾脏局限型 AIg 患者的死亡率不像心脏淀粉样变性患者那样高，但他们确实存在较高的发病率，生活质量也受到影响。发病机制的复杂性仍不完全清楚。已建立了一些模型来预测和评估对治疗的反应。免疫球蛋白轻链淀粉样变性导致的晚期肾功能不全患者的肾脏预后仍然较差，需要更好的治疗方法来保护肾功能。发生终末期肾病的患者可以接受肾移植进行肾脏替代治疗。
