Membranous nephropathy with systemic light-chain amyloidosis of remission after rituximab therapy:A case report

BACKGROUND About 70%-80%of patients with primary membranous nephropathy(MN)have phospholipase A2 receptor(PLA2R)in renal tissue.Systemic light-chain(AL)amyloidosis is the most common type of amyloidosis.MN complicated with amyloidosis is rare.CASE SUMMARY A 48-year-old Chinese male presented with nephrotic syndrome,positive serum PLA2R antibody,and positive serum and urine IgG-lambda type M-protein,with a normal ratio of serum-free light-chain level.The patient was diagnosed with MN accompanied by AL amyloidosis.He was treated with rituximab with glucocorticoids and CyBorD regimen of chemotherapy.After 21 mo of follow-up,the patient achieved complete remission regarding nephrotic syndrome without adverse effects of chemotherapy.CONCLUSION We report a case of PLA2R-related MN complicated with primary AL amyloidosis only with renal involvement and successfully treated with rituximab,glucocorticoids and chemotherapy.

皮肤脂肪及直肠黏膜活检对诊断系统性轻链淀粉样变性的意义

目的：分析我国汉族人群系统性轻链淀粉样变性（AL型淀粉样变性）的临床特征,比较不同部位活检对确诊的意义。方法：回顾性分析2009年1月至2014年10月在南京军区南京总医院肾脏科住院确诊的186例AL型淀粉样变性患者临床资料和病理诊断,以肾活检为诊断的金标准,比较皮肤脂肪活检和直肠黏膜活检对诊断AL型淀粉样变性的敏感性。结果：186例患者的平均年龄为（54.8±9.1）岁,男女比例为2.44∶1,143例（76.9%）的患者血清中检测出单克隆免疫球蛋白轻链。皮肤脂肪活检的诊断敏感性为92.5%,95%CI为88%～97%,直肠黏膜活检的诊断敏感性为94.9%,95%CI为92%～98%,结合皮肤脂肪和直肠黏膜两种活检方式,诊断阳性率达100%。结论：活检仍然是确诊AL型淀粉样变性的金标准,皮肤脂肪和直肠黏膜活检操作简单、花费低、并发症少且敏感性高,两种方法结合能够提高诊断的阳性率,对于难以进行肾活检的患者,皮肤脂肪和直肠黏膜是合适的活检取材部位。

膜性肾病合并系统性淀粉样变性

老年男性患者,临床表现为肾病综合征,肾活检证实为肾小球膜性肾病、肾间质淀粉样变性;皮肤脂肪组织淀粉样变性。最终诊断膜性肾病合并系统性淀粉样变性。