单克隆免疫球蛋白相关性肾小球疾病

Monoclonal immunoglobulin-related glomerular diseases

单克隆免疫球蛋白及其不同成分主要由克隆性增殖B细胞产生,可通过直接或间接损伤机制引起肾小球疾病。直接损伤机制包括增生性肾小球肾炎伴单克隆IgG沉积、纤维性肾小球肾炎和免疫管状肾小球病,间接机制包括C3肾病和血栓性微血管病。文章主要介绍这几类疾病的临床病理表现和治疗的最新进展。

Monoclonal immunoglobulin is the overproduction of an Ig by clonal expansion of cells from the B cell lineage. They can cause glomerular diseases through direct mechanism, including proliferative GN with monoclonal Ig deposits, fibrillary glomerulonephritis, and mmunotactoid glomerulopathy. Indirect injury mechanism includes C3 glomerulopathy and thrombotic microangiopathy. This review will provide an up-to-date understanding of these entities.