Department of Nephrology, Ziauddin University Hospital, Karachi, Pakistan.
Department of Nephrology, Liaquat National Hospital, Karachi, Pakistan.
Saudi J Kidney Dis Transpl. 2021 Jul-Aug;32(4):1166-1170. doi: 10.4103/1319-2442.338293.
About 3% of the population aged more than 50 years, is affected by monoclonal gammopathy of undetermined significance (MGUS), a premalignant condition that may progress to lymphoproliferative disorders. Since MGUS does not represent the diseases associated with end organ damage, a new term, monoclonal gammopathy of renal significance (MGRS) is coined for the monoclonal gammopathies that are associated with renal disorders. MGRS is classified into various types, including monoclonal immunoglobulin deposition disease (MIDD) and proliferative glomerulonephritis with monoclonal immunoglobulin deposition (PGNMID). PGNMID presents with membranoproliferative glomerulonephritis-type lesions associated with immunoglobulin deposition. This disease entity has a poor prognosis and its optimum treatment is yet to be established. We present the case of an elderly male, a known patient of light chain deposition disease, a form of MIDD, who initially lost to follow-up but later presented with PGNMID, wherein he was treated with steroid and cyclophosphamide, to which he responded.
约 3%的 50 岁以上人群患有意义未明的单克隆丙种球蛋白血症(MGUS),这是一种可能进展为淋巴增殖性疾病的癌前状态。由于 MGUS 不代表与终末器官损害相关的疾病,因此为与肾脏疾病相关的单克隆丙种球蛋白血症创造了一个新术语,即具有肾脏意义的单克隆丙种球蛋白血症(MGRS)。MGRS 分为多种类型,包括单克隆免疫球蛋白沉积病(MIDD)和伴有单克隆免疫球蛋白沉积的增殖性肾小球肾炎(PGNMID)。PGNMID 表现为与免疫球蛋白沉积相关的膜增殖性肾小球肾炎样病变。这种疾病实体预后不良,其最佳治疗方法尚未确定。我们报告了一名老年男性的病例,他是轻链沉积病(MIDD 的一种形式)的已知患者,最初失去了随访,但后来出现了 PGNMID,他接受了类固醇和环磷酰胺治疗,对此他有反应。