医学
肾小球肾炎
肾病综合征
肾病
单克隆
单克隆抗体
快速进行性肾小球肾炎
膜性肾病
免疫学
肾活检
病理
内科学
活检
抗体
肾
内分泌学
糖尿病
作者
Jin-Pu Li,Ya-Ting Du,Chuan Guo,Xiang-rong Rao,Li Shen
标识
DOI:10.1007/s40620-023-01583-2
摘要
We report the case of a 31-year-old male who presented with repeated episodes of nephritic-nephrotic syndrome in concomitance with infection. IgA was diagnosed and was initially responsive to treatment with immunosuppressors but further disease flare did not respond to treatment. Based on three consecutive renal biopsies over 8 years, a pattern switch from endocapillary proliferative IgA nephropathy to membranous proliferative glomerulonephritis with monoclonal IgAκ deposits was observed. Bortezomib-dexamethasone combination therapy finally led to a favorable renal response. This case provides new insights into the pathophysiological mechanisms of proliferative glomerulonephritis with monoclonal immunoglobin deposits (PGNMID), highlighting the importance of repeat renal biopsies and routine evaluation of monoclonal immunoglobin deposits in proliferative glomerulonephritis with refractory nephrotic syndrome.
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