Cryofibrinogen-associated glomerulonephritis diagnosed by mass spectrometry and immunoelectron microscopy

A 60-year-old male presented with accelerated hypertension, renal insufficiency, proteinuria, and hematuria. Percutaneous kidney biopsy revealed membranoproliferative glomerulonephritis (MPGN) without any immunoglobulin and complement deposition. On performing electron microscopy, deposits with a tu...

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Main Authors: Masanori Sudo (Author), Yuichi Sakamaki (Author), Michihiro Hosojima (Author), Suguru Yamamoto (Author), Yumi Ito (Author), Naofumi Imai (Author), Yoshikatsu Kaneko (Author), Shin Goto (Author), Chih-Ping Li (Author), Akira Shimizu (Author), Ichiei Narita (Author)
Format: Book
Published: Elsevier, 2019-03-01T00:00:00Z.
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Summary:A 60-year-old male presented with accelerated hypertension, renal insufficiency, proteinuria, and hematuria. Percutaneous kidney biopsy revealed membranoproliferative glomerulonephritis (MPGN) without any immunoglobulin and complement deposition. On performing electron microscopy, deposits with a tubular, organized structure and approximately 60 nm in diameter were detected in the glomerular subendothelial spaces and mesangial areas. Liquid chromatography-tandem mass spectrometry (LC-MS/MS) demonstrated the significantly increased deposition of fibrinogen and fibronectin in glomeruli. Immunohistochemistry and immunoelectron microscopy demonstrated that the deposits were composed of fibrinogen. Here we report a case of cryofibrinogen -associated GN in which LC-MS/MS and immunoelectron microscopy were useful for diagnosis. When MPGN with organized deposits without the deposition of immunoglobulins and complements is diagnosed, we considered the cryofibrinogen-associated GN in one of the differential diagnosis, and even skin symptoms cannot be detected. Keywords: Cryofibrinogen-related kidney disease, Liquid chromatography-tandem mass spectrometry, Immunoelectron microscopy
Item Description:2214-3300
10.1016/j.ehpc.2018.12.002