Abstract：
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.
摘要：
纤维性肾小球肾炎（FGN）是一种罕见的肾小球疾病，其特征是在肾小球系膜和肾小球基底膜中随机排列的原纤维沉积。临床特征包括大量白蛋白尿，血尿,高血压,和肾衰竭。通常,肾脏预后不好，大约50%的病例演变为终末期肾病。最近的蛋白质组学研究已经确定了热休克蛋白家族的成员，也称为DNAJB9，它沉积在FGN患者的肾小球中，在其他疾病中不存在，如淀粉样变性或免疫酸类肾小球病。这些发现是阐明该疾病发病机理的第一步，并可能有助于其诊断。因此，我们介绍了一例基线时伴有轻度白蛋白尿的FGN病例,并讨论了这一新型生物标志物对诊断该组患者的有用性.
