Department of Kidney Disease, Kawashima Hospital, Japan.
Department of Laboratory, Kawashima Hospital, Japan.
Intern Med. 2022 Jun 15;61(12):1863-1867. doi: 10.2169/internalmedicine.8258-21. Epub 2021 Nov 20.
A 39-year-old woman was hospitalized for nephrotic syndrome. Laboratory test results showed increased serum creatinine levels and urinary excretions of beta-2-microglobulin, and N-acetyl-beta-D-glucosaminidase. A renal biopsy revealed collapsing focal segmental glomerulosclerosis (FSGS) and acute interstitial nephritis. Despite treatment with pulse steroid followed by oral high-dose glucocorticoids and cyclosporines, heavy proteinuria persisted. After low-density lipoprotein apheresis (LDL-A) therapy was initiated, her proteinuria gradually decreased, leading to complete remission. A repeat renal biopsy after treatment revealed no collapsing glomeruli. Immediate LDL-A should be performed to treat cases of collapsing FSGS poorly responding to other treatments.
一位 39 岁女性因肾病综合征住院。实验室检查结果显示血清肌酐水平升高,β-2-微球蛋白和 N-乙酰-β-D-氨基葡萄糖苷酶尿排泄增加。肾活检显示局灶性节段性肾小球硬化(FSGS)伴急性间质性肾炎。尽管接受了脉冲类固醇治疗,随后又口服大剂量糖皮质激素和环孢菌素治疗,但仍持续存在大量蛋白尿。开始进行低密度脂蛋白吸附(LDL-A)治疗后,患者的蛋白尿逐渐减少,病情完全缓解。治疗后重复肾活检显示没有塌陷的肾小球。对于其他治疗反应不佳的塌陷 FSGS 病例,应立即进行 LDL-A 治疗。