摘要
微纖維性腎絲球腎炎在臨床上罕見，病人通常以大量蛋白尿、血尿、高血壓及腎功能異常表現。其特徵是電子顯微鏡下呈現20 nm大小的微纖維沉積於腎絲球系膜及基底膜，這種微纖維沉積需與類澱粉沉積症或類免疫球性腎絲球腎炎做鑑別診斷。最近DNAJB9新型免疫組織化學生物標誌物對纖維性腎小球腎炎具高敏感和特異性，可以更快速地診斷這種疾病。微纖維性腎絲球腎炎致病機轉至今不明，尚無有效的治療藥物，且約一半的病患在4～5年後將惡化成末期腎病變。透析或腎臟移植皆為腎衰竭後的治療選擇，腎移植後的復發也曾被報導。
並列摘要
Fibrillary glomerulonephritis is a uncommon cause of glomerular disease. Patient often presented with heavy proteinuria, followed by hematuria, hypertension, renal insulficiency. The pathognomonic changes of pathology are seen on electron microscopy, which showed random fibrillar deposit, size about 20 nm within mesangium and glomerular basement membrane. The diagnosis should be differentiated from amyloidosis or immunotactoid glomerulonephritis. Furthermore, DNAJB9 novel immunohistochemical biomarker, which is sensitive and specific for fibrillary glomerulonephritis allows more rapid diagnosis of this disease. The etiology is still unknown and there is no effective pharmacologic treatment. About half of patients may develop end stage kidney disease 4-5 years after diagnosis. Dialysis or kidney transplant are rational treatment options for renal failure, but recurrence of primary disease had also been reported.