Wen Jiqiu, Wang Wei, Xu Feng, Chen Jinsong, Zhang Mingchao, Cheng Dongrui, Ni Xuefeng, Li Xue, Liu Zhihong
National Clinical Research Center of Kidney Diseases, Jinling Hospital, Nanjing University Jinling School of Medicine, Nanjing, 210000, China.
National Clinical Research Center of Kidney Diseases, Jinling Hospital, Nanjing Medical University, Nanjing, 210000, China.
BMC Nephrol. 2018 Jul 11;19(1):173. doi: 10.1186/s12882-018-0969-3.
We present a case series of 5 patients with proliferative glomerulonephritis with monoclonal IgG deposits (PGNMID) of renal allografts to better define its natural history, presenting characteristics, pathological features and treatment outcome.
These 5 patients presented 5 to 19 months post-kidney transplantation for complaints of serum creatinine (Scr) elevation, proteinuria or hematuria. Membranoproliferative glomerulonephritis (MPGN) pattern was the most frequently observed histological manifestation. Immunofluorescence showed monoclonal IgG3κin 3 patients and IgG3λ in the other 2 cases. Immunofluorescence staining helped to establish PGNMID in the absence of conspicuous microscopic changes in one case. Rituximab and bortezomib were effective in alleviating proteinuria in all 4 treated patients and decreasing Scr in 2 cases. Plasmapheresis treatment in another patient was not effective in preventing Scr elevation. At last-follow-up, 2 patients were in dialysis and 2 had improved kidney function with almost normal Scr and no proteinuria. The remaining one patient died of pulmonary infections.
We conclude that PGNMID occurs early after kidney transplant. PGNMID should be included in the differential diagnoses of recurrent MPGN in renal allografts. Rituximab and bortezomib are helpful to decrease proteinuria and Scr in a subset of patients. Larger studies are needed to conclusively establish best treatment strategies for PGNMID in renal allografts.
我们报告了一组5例肾移植受者发生的伴有单克隆IgG沉积的增殖性肾小球肾炎（PGNMID）病例，以更好地明确其自然病程、临床表现、病理特征及治疗结果。
这5例患者在肾移植后5至19个月出现血清肌酐（Scr）升高、蛋白尿或血尿等症状。膜增生性肾小球肾炎（MPGN）型是最常见的组织学表现。免疫荧光显示3例患者为单克隆IgG3κ，另外2例为IgG3λ。免疫荧光染色在1例显微镜下无明显改变的病例中有助于确诊PGNMID。利妥昔单抗和硼替佐米在所有4例接受治疗的患者中均有效减轻蛋白尿，2例患者Scr降低。另一例患者行血浆置换治疗未能有效预防Scr升高。末次随访时，2例患者接受透析治疗，2例患者肾功能改善，Scr几乎正常且无蛋白尿。其余1例患者死于肺部感染。
我们得出结论，PGNMID在肾移植后早期发生。PGNMID应纳入肾移植中复发性MPGN的鉴别诊断。利妥昔单抗和硼替佐米有助于部分患者降低蛋白尿和Scr。需要开展更大规模的研究以最终确定肾移植中PGNMID的最佳治疗策略。
