AL Amyloidosis Presenting With Crescentic Glomerulonephritis

Ann A. Wang; Yashpal S. Kanwar; Vikram Aggarwal; Anand Srivastava

doi:10.1016/j.xkme.2021.02.009

AL Amyloidosis Presenting With Crescentic Glomerulonephritis

Ann A. Wang, Yashpal S. Kanwar, Vikram Aggarwal, Anand Srivastava^*

^*Corresponding author for this work

Research output: Contribution to journal › Article › peer-review

1 Scopus citations

Abstract

Kidney amyloidosis typically presents with nephrotic-range proteinuria. Rare cases of crescentic glomerulonephritis have been reported in patients with kidney amyloidosis but most cases were in the setting of patients with AA amyloidosis from long-standing inflammation and malignancy. We present a case of a previously healthy man in his 70s who was admitted with severe acute kidney injury, nephrotic-range proteinuria, and nephritic urinary sediment. Initial serologic testing for causes of rapidly progressive glomerulonephritis were negative. Kidney biopsy demonstrated the presence of active cellular and fibrocellular crescents with Congo red–positive staining in glomeruli and microvasculature on light microscopy and amyloid fibrils in glomerular basement membrane on electron microscopy. Urinary protein electrophoresis revealed monoclonal λ light chains, leading to a diagnosis of kidney AL amyloidosis, which was confirmed with bone marrow biopsy. Our case illustrates that AL amyloidosis can present with findings suspicious for rapidly progressive glomerulonephritis and crescent formation on kidney biopsy specimens.

Original language	English (US)
Pages (from-to)	644-648
Number of pages	5
Journal	Kidney Medicine
Volume	3
Issue number	4
DOIs	https://doi.org/10.1016/j.xkme.2021.02.009
State	Published - Jul 1 2021

Funding

Dr Kanwar is supported by National Institutes of Health (NIH) grant R01DK060635. Dr Srivastava is supported by NIH grant K23DK120811 and core resources from the George M. O’Brien Kidney Research Center at Northwestern University (NU-GoKIDNEY) P30DK114857. Ann A. Wang, MD, Yashpal S. Kanwar, MD, PhD, Vikram Aggarwal, MD, and Anand Srivastava, MD, MPH. Dr Kanwar is supported by National Institutes of Health (NIH) grant R01DK060635. Dr Srivastava is supported by NIH grant K23DK120811 and core resources from the George M. O'Brien Kidney Research Center at Northwestern University (NU-GoKIDNEY) P30DK114857. Dr Srivastava received honoraria from Horizon Therapeutics, PLC, and AstraZeneca and consulting fees from CVS Caremark. The remaining authors declare that they have no relevant financial interests. The authors declare that they have obtained consent, from a relative with appropriate authority, for publication of the information about the patient reported in this article and any associated supplementary material. Received September 14, 2020. Evaluated by 3 external peer reviewers, with direct editorial input from an Associate Editor and the Editor-in-Chief. Accepted in revised form February 15, 2021.

Keywords

AL amyloidosis
glomerular disease
kidney amyloidosis
kidney biopsy
rapidly progressive glomerulonephritis

ASJC Scopus subject areas

Nephrology
Internal Medicine

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10.1016/j.xkme.2021.02.009

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title = "AL Amyloidosis Presenting With Crescentic Glomerulonephritis",

abstract = "Kidney amyloidosis typically presents with nephrotic-range proteinuria. Rare cases of crescentic glomerulonephritis have been reported in patients with kidney amyloidosis but most cases were in the setting of patients with AA amyloidosis from long-standing inflammation and malignancy. We present a case of a previously healthy man in his 70s who was admitted with severe acute kidney injury, nephrotic-range proteinuria, and nephritic urinary sediment. Initial serologic testing for causes of rapidly progressive glomerulonephritis were negative. Kidney biopsy demonstrated the presence of active cellular and fibrocellular crescents with Congo red–positive staining in glomeruli and microvasculature on light microscopy and amyloid fibrils in glomerular basement membrane on electron microscopy. Urinary protein electrophoresis revealed monoclonal λ light chains, leading to a diagnosis of kidney AL amyloidosis, which was confirmed with bone marrow biopsy. Our case illustrates that AL amyloidosis can present with findings suspicious for rapidly progressive glomerulonephritis and crescent formation on kidney biopsy specimens.",

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author = "Wang, {Ann A.} and Kanwar, {Yashpal S.} and Vikram Aggarwal and Anand Srivastava",

note = "Funding Information: Dr Kanwar is supported by National Institutes of Health (NIH) grant R01DK060635. Dr Srivastava is supported by NIH grant K23DK120811 and core resources from the George M. O{\textquoteright}Brien Kidney Research Center at Northwestern University (NU-GoKIDNEY) P30DK114857. Funding Information: Ann A. Wang, MD, Yashpal S. Kanwar, MD, PhD, Vikram Aggarwal, MD, and Anand Srivastava, MD, MPH. Dr Kanwar is supported by National Institutes of Health (NIH) grant R01DK060635. Dr Srivastava is supported by NIH grant K23DK120811 and core resources from the George M. O'Brien Kidney Research Center at Northwestern University (NU-GoKIDNEY) P30DK114857. Dr Srivastava received honoraria from Horizon Therapeutics, PLC, and AstraZeneca and consulting fees from CVS Caremark. The remaining authors declare that they have no relevant financial interests. The authors declare that they have obtained consent, from a relative with appropriate authority, for publication of the information about the patient reported in this article and any associated supplementary material. Received September 14, 2020. Evaluated by 3 external peer reviewers, with direct editorial input from an Associate Editor and the Editor-in-Chief. Accepted in revised form February 15, 2021. Publisher Copyright: {\textcopyright} 2021 The Authors",

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N2 - Kidney amyloidosis typically presents with nephrotic-range proteinuria. Rare cases of crescentic glomerulonephritis have been reported in patients with kidney amyloidosis but most cases were in the setting of patients with AA amyloidosis from long-standing inflammation and malignancy. We present a case of a previously healthy man in his 70s who was admitted with severe acute kidney injury, nephrotic-range proteinuria, and nephritic urinary sediment. Initial serologic testing for causes of rapidly progressive glomerulonephritis were negative. Kidney biopsy demonstrated the presence of active cellular and fibrocellular crescents with Congo red–positive staining in glomeruli and microvasculature on light microscopy and amyloid fibrils in glomerular basement membrane on electron microscopy. Urinary protein electrophoresis revealed monoclonal λ light chains, leading to a diagnosis of kidney AL amyloidosis, which was confirmed with bone marrow biopsy. Our case illustrates that AL amyloidosis can present with findings suspicious for rapidly progressive glomerulonephritis and crescent formation on kidney biopsy specimens.

AB - Kidney amyloidosis typically presents with nephrotic-range proteinuria. Rare cases of crescentic glomerulonephritis have been reported in patients with kidney amyloidosis but most cases were in the setting of patients with AA amyloidosis from long-standing inflammation and malignancy. We present a case of a previously healthy man in his 70s who was admitted with severe acute kidney injury, nephrotic-range proteinuria, and nephritic urinary sediment. Initial serologic testing for causes of rapidly progressive glomerulonephritis were negative. Kidney biopsy demonstrated the presence of active cellular and fibrocellular crescents with Congo red–positive staining in glomeruli and microvasculature on light microscopy and amyloid fibrils in glomerular basement membrane on electron microscopy. Urinary protein electrophoresis revealed monoclonal λ light chains, leading to a diagnosis of kidney AL amyloidosis, which was confirmed with bone marrow biopsy. Our case illustrates that AL amyloidosis can present with findings suspicious for rapidly progressive glomerulonephritis and crescent formation on kidney biopsy specimens.

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AL Amyloidosis Presenting With Crescentic Glomerulonephritis

Abstract

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Keywords

ASJC Scopus subject areas

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