Nephrology Section, Hospital Universitario Príncipe de Asturias, Alcalá de Henares, Madrid, Spain.
Saudi J Kidney Dis Transpl. 2023 Sep 1;34(5):458-461. doi: 10.4103/1319-2442.397209. Epub 2024 Mar 11.
Fibrillary glomerulonephritis (FGN) is a rare glomerular disorder characterized by the deposition of randomly arranged fibrils in the mesangium and the glomerular basement membrane. Clinical features include massive albuminuria, hematuria, high blood pressure, and kidney failure. Usually, the renal prognosis is not favorable, with evolution to end-stage renal disease in approximately 50% of cases. Recent studies in proteomics have identified a member of the heat shock protein family, also called DNAJB9, which is deposited in the glomerulus of patients with FGN and is not present in other diseases, such as amyloidosis or immunotactoid glomerulopathy. These findings are the first step to clarify the pathogenesis of this disease and could facilitate its diagnosis. Hence, we present a case of FGN with mild albuminuria at baseline and discuss the usefulness of this novel biomarker for diagnosing this group of patients.
纤维状肾小球肾炎(FGN)是一种罕见的肾小球疾病,其特征是在系膜和肾小球基底膜中沉积排列不规则的纤维。临床特征包括大量蛋白尿、血尿、高血压和肾衰竭。通常,肾脏预后不佳,约 50%的病例会发展为终末期肾病。最近的蛋白质组学研究鉴定了热休克蛋白家族的一个成员,也称为 DNAJB9,它在 FGN 患者的肾小球中沉积,而在其他疾病(如淀粉样变性或免疫触须状肾小球病)中不存在。这些发现是阐明该疾病发病机制的第一步,也有助于其诊断。因此,我们报告了一例基线时仅有轻度蛋白尿的 FGN,并讨论了这种新型生物标志物在诊断该组患者中的作用。