Takehara Eriko, Mandai Shintaro, Shikuma Satomi, Akita Wataru, Chiga Motoko, Mori Takayasu, Oda Takashi, Kuwahara Michio, Uchida Shinichi
Department of Nephrology, Shuuwa General Hospital, Japan.
Intern Med. 2017;56(7):811-817. doi: 10.2169/internalmedicine.56.7778. Epub 2017 Apr 1.
A 55-year-old man developed rapidly progressive glomerulonephritis and nephrotic syndrome. A kidney biopsy specimen showed diffuse proliferative and crescentic glomerulonephritis with monoclonal IgG1κ, humps, and nephritis-associated plasmin receptor, indicating infection-associated proliferative glomerulonephritis with monoclonal immunoglobulin G deposits (PGNMID). Despite dialysis-dependent renal failure, symptomatic therapy resulted in spontaneous recovery of the renal function, mimicking post-infectious glomerulonephritis (PIGN). A heterozygous complement factor H mutation was detected by comprehensive genetic testing of alternative pathway regulatory genes, which might lead to persistent infection-triggered alternative pathway activation and account for severe glomerulonephritis. Post-infectious PGNMID and PIGN might share common clinical presentations and pathogenesis related to the complement pathway.
一名55岁男性患上了快速进展性肾小球肾炎和肾病综合征。肾活检标本显示弥漫性增生性和新月体性肾小球肾炎，伴有单克隆IgG1κ、驼峰和肾炎相关纤溶酶受体，提示感染相关的增生性肾小球肾炎伴单克隆免疫球蛋白G沉积（PGNMID）。尽管出现了依赖透析的肾衰竭，但对症治疗使肾功能自发恢复，类似于感染后肾小球肾炎（PIGN）。通过对替代途径调节基因进行全面基因检测，发现了一个杂合的补体因子H突变，这可能导致感染引发的替代途径持续激活，并解释严重肾小球肾炎的原因。感染后PGNMID和PIGN可能具有与补体途径相关的共同临床表现和发病机制。
