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Published on: November 1, 2018
Renal-Limited Cryofibrinogen-Associated Glomerulonephritis Diagnosed Using Electron Microscopy
Shoko Ochiai1, Akihiro Minakawa1, Shuichi Hisanaga2
1Division of Cardiovascular Medicine and Nephrology, Department of Internal Medicine, Faculty of Medicine, University of Miyazaki, Miyazaki, Japan.
Abstract:
Cryofibrinogen-associated glomerulonephritis (CryoFiGN) is a rare, diagnostically challenging cause of nephrotic syndrome, and its clinical presentation can vary considerably. Herein, we report the case of a 76-year-old man who presented with nephrotic syndrome; biopsy showed a C3-dominant membranoproliferative pattern of injury. Electron microscopy revealed organised subendothelial microtubular deposits (40-80 nm), confirming CryoFiGN, despite inconclusive results on mass spectrometry. The patient progressed to end-stage kidney disease and later developed systemic vasculitis with skin ulcers and gastrointestinal bleeding. CryoFiGN may initially manifest as a renal-limited disease and evolve into systemic vasculitis. Electron microscopy is pivotal for diagnosis when proteomic analysis is inconclusive and long-term surveillance is warranted.

