Rosenstock Jordan L, Markowitz Glen S
Division of Nephrology, Lenox Hill Hospital, Hofstra Northwell School of Medicine, New York, New York, USA.
Department of Pathology and Cell Biology, Columbia University Irving Medical Center, New York, New York, USA.
Kidney Int Rep. 2019 Apr 29;4(7):917-922. doi: 10.1016/j.ekir.2019.04.013. eCollection 2019 Jul.
Fibrillary glomerulonephritis (FGN) is a rare proliferative form of glomerular disease characterized by randomly oriented fibrillar deposits with a mean diameter of 20 nm. By immunofluorescence (IF), the deposits stain for IgG, C3, and κ and λ light chains, suggesting that the fibrils may be composed of antigen-antibody immune complexes. A recent major advance in our understanding of the pathogenesis of FGN resulted from the discovery that a major component of the fibrils is DNA-J heat-shock protein family member B9 (DNAJB9), and immunohistochemical staining for DNAJB9 now makes it possible to diagnose FGN in the absence of ultrastructural evaluation. FGN has a poor prognosis, treatment options are currently limited, and transplant recurrence is not uncommon.
纤维性肾小球肾炎（FGN）是一种罕见的增生性肾小球疾病，其特征是存在平均直径为20纳米的随机排列的纤维状沉积物。通过免疫荧光（IF）检查，沉积物对IgG、C3以及κ和λ轻链呈阳性染色，提示这些纤维可能由抗原 - 抗体免疫复合物组成。我们对FGN发病机制认识的一项近期重大进展源于发现纤维的主要成分是DNA - J热休克蛋白家族成员B9（DNAJB9），现在针对DNAJB9的免疫组织化学染色使得在无需超微结构评估的情况下诊断FGN成为可能。FGN预后较差，目前治疗选择有限，移植后复发并不罕见。
