Shah H H, Thakkar J, Pullman J M, Mathew A T
Department of Medicine, Division of Kidney Diseases and Hypertension, North Shore University Hospital and Long Island Jewish Medical Center, Hofstra Northwell School of Medicine, Great Neck, USA.
Department of Pathology, Montefiore Medical Center, Bronx, New York, USA.
Indian J Nephrol. 2017 Mar-Apr;27(2):157-160. doi: 10.4103/0971-4065.200521.
Fibrillary glomerulonephritis (FGN) is a rare primary glomerular disease that commonly presents clinically with hypertension, proteinuria, microscopic hematuria, and varying degree of renal insufficiency. Histologically, FGN can present with different patterns of glomerular injury, more commonly mesangioproliferative, membranoproliferative, and membranous nephropathy. While crescent formation has been described in some kidney biopsy series of FGN, crescentic glomerulonephritis pattern of glomerular injury has been rarely described. Optimal therapy and outcomes in FGN presenting with crescentic GN is not currently known. We report an adult patient who presented with massive proteinuria and severe renal failure. The kidney biopsy revealed crescentic FGN (C-FGN). The patient remained dialysis dependent despite immunosuppressive therapy. We also briefly review FGN, and the few reported cases of C-FGN that presented as rapidly progressive or advanced renal failure in the literature.
纤维性肾小球肾炎（FGN）是一种罕见的原发性肾小球疾病，临床上通常表现为高血压、蛋白尿、镜下血尿以及不同程度的肾功能不全。在组织学上，FGN可呈现不同类型的肾小球损伤模式，较为常见的是系膜增生性、膜增生性和膜性肾病。虽然在一些FGN的肾活检系列中曾描述过新月体形成，但肾小球损伤的新月体性肾小球肾炎模式却鲜有报道。目前尚不清楚伴有新月体性肾小球肾炎的FGN的最佳治疗方法及治疗效果。我们报告了一名成年患者，该患者表现为大量蛋白尿和严重肾衰竭。肾活检显示为新月体性FGN（C-FGN）。尽管接受了免疫抑制治疗，该患者仍依赖透析。我们还简要回顾了FGN以及文献中报道的少数几例表现为快速进展性或晚期肾衰竭的C-FGN病例。
