Allinovi Marco, Aterini Lorenzo, Caroti Leonardo, Antognoli Giulia, Cirami Calogero Lino
Nephrology, Dialysis and Transplantation Unit, Careggi University Hospital, Largo Brambilla, 3, 50134, Florence, Italy.
Department of Biomedical Experimental and Clinical Sciences "Mario Serio", University of Florence, Florence, Italy.
J Nephrol. 2025 Mar;38(2):541-550. doi: 10.1007/s40620-024-02160-x. Epub 2024 Dec 17.
About 4-7% of renal biopsies show a monoclonal gammopathy-related nephropathy, such as AL amyloidosis, cast nephropathy, or light chain deposition disease. Both a high prevalence and a causal role of monoclonal gammopathy have been observed in patients with C3 glomerulopathy or thrombotic microangiopathy, although a definitive causative role cannot be established in most cases (potentially monoclonal gammopathy-related nephropathies). A coexisting monoclonal gammopathy has been identified in many cases of nephropathy without a defined causative role (monoclonal gammopathy-unrelated nephropathies). The aim of this study was to investigate the prevalence and distribution of monoclonal gammopathy in patients who underwent a renal biopsy and assess its possible causal role in nephropathies not ordinarily related to monoclonal gammopathy.
In our single-center retrospective observational study, we considered patients who underwent native kidney biopsy from 2009 to 2023 at the Nephrology Unit, Careggi University Hospital, Florence (Italy) and for whom a complete monoclonal gammopathy workup (serum electrophoresis, serum and urinary immunofixation, serum free light chains) was available.
Overall, 827 patients were included: 208 (25%) had a monoclonal gammopathy: in 104 cases the monoclonal gammopathy was unrelated to the kidney disease; 87 subjects showed renal pathology related to monoclonal gammopathy (monoclonal gammopathy-related nephropathies). Patients with thrombotic microangiopathy and C3 glomerulopathy (potentially monoclonal gammopathy-related nephropathies) exhibited a prevalence of monoclonal gammopathy > 30%. In a subgroup of diagnoses (e.g. tubulointerstitial nephritis, membranoproliferative glomerulonephritis) a possible causal and/or prognostic role of a concomitant monoclonal gammopathy may be hypothesized.
In our cohort, one fourth of patients undergoing a renal biopsy had a monoclonal gammopathy, although in half of them the monoclonal gammopathy did not have a causative role in the kidney disease. Hence, it is impossible to conclude that a monoclonal gammopathy in the context of renal disease equates to a causal association without performing a renal biopsy because of the high frequency of monoclonal gammopathy in patients undergoing a kidney biopsy.
约4%-7%的肾活检显示为单克隆丙种球蛋白病相关肾病,如AL淀粉样变性、管型肾病或轻链沉积病。在C3肾小球病或血栓性微血管病患者中已观察到单克隆丙种球蛋白病的高患病率及其因果作用,尽管在大多数情况下(可能是单克隆丙种球蛋白病相关肾病)无法确定明确的因果关系。在许多无明确病因的肾病病例(单克隆丙种球蛋白病无关肾病)中已发现并存的单克隆丙种球蛋白病。本研究的目的是调查接受肾活检患者中单克隆丙种球蛋白病的患病率和分布情况,并评估其在通常与单克隆丙种球蛋白病无关的肾病中的可能因果作用。
在我们的单中心回顾性观察研究中,我们纳入了2009年至2023年在意大利佛罗伦萨卡雷吉大学医院肾病科接受自体肾活检且有完整单克隆丙种球蛋白病检查(血清电泳、血清和尿免疫固定电泳、血清游离轻链)结果的患者。
总共纳入了827例患者:208例(25%)患有单克隆丙种球蛋白病:其中104例单克隆丙种球蛋白病与肾脏疾病无关;87例患者表现出与单克隆丙种球蛋白病相关的肾脏病理改变(单克隆丙种球蛋白病相关肾病)。血栓性微血管病和C3肾小球病患者(可能是单克隆丙种球蛋白病相关肾病)中单克隆丙种球蛋白病的患病率>30%。在一个诊断亚组(如肾小管间质性肾炎、膜增生性肾小球肾炎)中,可以推测并存的单克隆丙种球蛋白病可能具有因果和/或预后作用。
在我们的队列中,接受肾活检的患者中有四分之一患有单克隆丙种球蛋白病,尽管其中一半患者的单克隆丙种球蛋白病在肾脏疾病中没有因果关系。因此,由于接受肾活检的患者中单克隆丙种球蛋白病的发生率很高,在未进行肾活检的情况下,无法得出肾病背景下的单克隆丙种球蛋白病等同于因果关联的结论。