Inoue Reiko, Fujigaki Yoshihide, Kobayashi Kana, Tamura Yoshifuru, Ota Tatsuru, Shibata Shigeru, Ishida Tsuyoshi, Kondo Fukuo, Yamaguchi Yutaka, Uchida Shunya
Department of Internal Medicine, Teikyo University School of Medicine, Japan.
Intern Med. 2017;56(4):419-423. doi: 10.2169/internalmedicine.56.7548. Epub 2017 Feb 15.
We report a 70-year-old woman with Sjögren's syndrome who had severe renal dysfunction with mild proteinuria and elevated urinary low-molecular-weight proteins. Based on these clinical presentations, interstitial nephritis due to Sjögren's syndrome was strongly suspected. Unexpectedly, renal pathology revealed amyloid light-chain (AL) lambda-type depositions predominantly in the vasculatures with severe tubulointerstitial damage. Concentrated urine immunofixation was positive for Bence Jones lambda-type monoclonal proteins. Given the involvement in other organs, systemic AL amyloidosis was diagnosed. The patient underwent chemotherapy, but hemodialysis was ultimately instituted. It should be remembered that renal amyloidosis occurs as a clinical presentation of interstitial nephritis.
我们报告了一名70岁患有干燥综合征的女性，她有严重的肾功能不全，伴有轻度蛋白尿和尿中低分子量蛋白升高。基于这些临床表现，强烈怀疑是干燥综合征所致的间质性肾炎。出乎意料的是，肾脏病理显示淀粉样轻链（AL）λ型沉积主要在血管中，伴有严重的肾小管间质损伤。浓缩尿免疫固定电泳显示本-周氏λ型单克隆蛋白阳性。鉴于其他器官也有受累，诊断为系统性AL淀粉样变性。患者接受了化疗，但最终还是进行了血液透析。应该记住，肾淀粉样变性可表现为间质性肾炎。
