Nephrology Unit , Hiratsuka Kyosai Hospital , Hiratsuka , Japan.
Department of Pathology , Yokohama City University School of Medicine , Yokohama , Japan.
Clin Kidney J. 2013 Dec;6(6):635-8. doi: 10.1093/ckj/sft121. Epub 2013 Oct 6.
Several types of glomerulonephritis associated with dysproteinemia, such as AL-amyloidosis, light- and heavy-chain deposition disease, and type 1 cryoglobulinemic glomerulopathy, demonstrate monoclonal immunoglobulin deposition. Progressive glomerulonephritis with monoclonal IgG deposits (PGNMID) is also known to feature monoclonal glomerular deposits, but most of these cases occur without underlying disease. We here report a case of recurrent PGNMID that developed as diffuse cellular crescentic glomerulonephritis 8 years after an initial diagnosis of membranoproliferative glomerulonephritis (MPGN). Determination of the monoclonality of the deposited immunoglobulin is vital to make the correct diagnosis and enable an early administration of aggressive immunosuppressive therapy.
几种与蛋白尿相关的肾小球肾炎与单克隆免疫球蛋白沉积有关，如 AL 淀粉样变性、轻链和重链沉积病以及 1 型冷球蛋白血症性肾小球病，均显示单克隆免疫球蛋白沉积。伴有单克隆 IgG 沉积的进行性肾小球肾炎（PGNMID）也以单克隆肾小球沉积为特征，但这些病例大多发生于无基础疾病的情况下。我们在此报告一例复发性 PGNMID，其在初次诊断为膜增生性肾小球肾炎（MPGN） 8 年后发展为弥漫性细胞性新月体肾小球肾炎。确定沉积免疫球蛋白的单克隆性对于正确诊断和早期进行积极的免疫抑制治疗至关重要。
