具有肾脏意义的单克隆免疫球蛋白病的发病机制研究

Study on the pathogenesis of monoclonal gammopathy of renal significance

具有肾脏意义的单克隆免疫球蛋白病(monoclonal gammopathy of renal significance,MGRS)表现为不同肾脏损害的一组疾病,是一种新的肾病类型,且病种多种多样,疾病机制复杂。当MGRS时,由于B淋巴或浆细胞克隆性增殖,出现大量单克隆免疫球蛋白(monoclonal immunoglobulin,MIg)和/或大量的游离轻链(free light chain,FLC)。完整的MIg可与肾小球的固有细胞相互作用改变以改变其生物学并促进肾脏疾病发展,但单克隆FLC可潜在地改变整个肾单位中各种细胞的功能。鉴于MIg和单克隆FLC与MGRS的关系,抑制MIg和单克隆FLC将是一种有前景的治疗MGRS的方法。该文主要从肾脏受累部位即肾小球、肾小管-间质、肾血管对MGRS的发病机制作一综述。

Monoclonal gammopathy of renal significance(MGRS) is a group of diseases with different renal damage. It is a new type of renal disease with various types of diseases and complex disease mechanism. In MGRS, due to the clonal proliferation of B lymphoid cells or plasma cells, a large number of monoclonal immunoglobulin(MIg) and/or a large number of free light chain(FLC) appear. Intact MIg can interact with intrinsic cells of glomerulus to change its biology in order to promote the development of renal disease, while monoclonal FLC can potentially alter the function of various cells throughout the nephron. Given the relationship of MIg and monoclonal FLC to MGRS, inhibition of MIg and monoclonal FLC would be a promising approach for the treatment of MGRS. This paper reviews the pathogenesis of MGRS from the sites of renal involvement, including glomerulus, renal tubule-interstitium and renal blood vessel.