Cryofibrinogen-Associated Glomerulonephritis

Sethi, Sanjeev; Yachoui, Ralph; Murray, David; Alexander, Mariam P.

doi:10.1053/j.ajkd.2016.08.031

Cited by 13 publications

(22 citation statements)

References 24 publications

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“…However, ultrastructural examination revealed unusual subendothelial deposits that were similar to those of the two patients reported by Sethi et al [6]. We therefore suspected a diagnosis of cryofibrinogen-associated glomerulonephritis.…”

Section: Case Presentationsupporting

confidence: 84%

“…The mean diameter of the fibrils was 185 nm (range, 150-220 nm). These deposits resembled those described in two patients diagnosed with cryofibrinogen-associated glomerulonephritis [6]. In addition, diffuse foot process effacement and a mild increase in mesangial matrix were present.…”

Section: Case Presentationsupporting

confidence: 79%

“…4). These substances have been detected in patients previously reported as having cryofibrinogenassociated glomerulonephritis [6,7]. IgG1, IgA1, and kappa light chain were also detected at levels comparable to those in a control.…”

Section: Case Presentationsupporting

confidence: 64%

“…Although the kidney can also be a target organ, there are few reports describing the renal pathology of cryofibrinogenemia-related glomerulopathy [4][5][6][7]. Sethi et al described two cases [6] and Sudo et al one case of cryofibrinogen-associated glomerulonephritis [7]. Organized deposits were observed in the subendothelial area in all three cases.…”

Section: Introductionmentioning

confidence: 86%

“…The skin is usually the target organ: manifestations including purpura, livedo reticularis, Raynaud's phenomenon, ulceration, and gangrene [3]. Although the kidney can also be a target organ, there are few reports describing the renal pathology of cryofibrinogenemia-related glomerulopathy [4][5][6][7]. Sethi et al described two cases [6] and Sudo et al one case of cryofibrinogen-associated glomerulonephritis [7].…”

Section: Introductionmentioning

confidence: 99%

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Characteristic electron-microscopic features of cryofibrinogen-associated glomerulonephritis: a case report

et al. 2020

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Background: Cryofibrinogenemia is a rare disorder that mainly affects the skin and occasionally the kidney. However, there are few published reports of cryofibrinogenemia-associated renal pathology. We therefore report a patient with cryofibrinogen-associated glomerulonephritis. Samples from this patient were examined by electron microscopy, laser microdissection, and liquid chromatography-tandem mass spectrometry (LC-MS/MS). Case presentation: A 78-year-old Japanese man presented with declining renal function, proteinuria, and gross hematuria. Kidney biopsy showed a membranoproliferative pattern with crescent formation and dominant C3c deposition in which subendothelial deposits with uniquely organized electron-microscopic features were observed. Additional ultrastructural analysis of cryoprecipitates extracted from plasma revealed similar structures of the glomerular subendothelial deposits. LC-MS/MS identified an increase in fibrinogen α, β, and γ chains, fibronectin, filamin-A, and C3. The glomerular lesions were diagnosed as cryofibrinogen-associated glomerulonephritis on the basis of these findings. Conclusions: Although there are few reports of cryofibrinogen-associated glomerulonephritis, we believe that accurate diagnosis can be achieved by performing LC-MS/MS and ultrastructural analysis.

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Section: Case Presentationsupporting

confidence: 84%

Section: Case Presentationsupporting

confidence: 79%

Section: Case Presentationsupporting

confidence: 64%