AL Amyloidosis Presenting With Crescentic Glomerulonephritis
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 c...
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doaj-8b0e50a32cff4b0880f61153930795742021-08-04T04:20:32ZengElsevierKidney Medicine2590-05952021-07-0134644648AL Amyloidosis Presenting With Crescentic GlomerulonephritisAnn A. Wang0Yashpal S. Kanwar1Vikram Aggarwal2Anand Srivastava3Graduate Medical Education, Northwestern University Feinberg School of Medicine, Chicago, ILDepartment of Pathology, Northwestern University Feinberg School of Medicine, Chicago, ILDivision of Nephrology and Hypertension, Northwestern University Feinberg School of Medicine, Chicago, ILDivision of Nephrology and Hypertension, Northwestern University Feinberg School of Medicine, Chicago, IL; Center for Translational Metabolism and Health, Institute for Public Health and Medicine, Northwestern University Feinberg School of Medicine, Chicago, IL; Address for Correspondence: Anand Srivastava, MD, MPH, 633 N St Clair St, Ste 18-083, Chicago, IL 60611.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.http://www.sciencedirect.com/science/article/pii/S2590059521000741AL amyloidosiskidney amyloidosisrapidly progressive glomerulonephritisglomerular diseasekidney biopsy |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Ann A. Wang Yashpal S. Kanwar Vikram Aggarwal Anand Srivastava |
spellingShingle |
Ann A. Wang Yashpal S. Kanwar Vikram Aggarwal Anand Srivastava AL Amyloidosis Presenting With Crescentic Glomerulonephritis Kidney Medicine AL amyloidosis kidney amyloidosis rapidly progressive glomerulonephritis glomerular disease kidney biopsy |
author_facet |
Ann A. Wang Yashpal S. Kanwar Vikram Aggarwal Anand Srivastava |
author_sort |
Ann A. Wang |
title |
AL Amyloidosis Presenting With Crescentic Glomerulonephritis |
title_short |
AL Amyloidosis Presenting With Crescentic Glomerulonephritis |
title_full |
AL Amyloidosis Presenting With Crescentic Glomerulonephritis |
title_fullStr |
AL Amyloidosis Presenting With Crescentic Glomerulonephritis |
title_full_unstemmed |
AL Amyloidosis Presenting With Crescentic Glomerulonephritis |
title_sort |
al amyloidosis presenting with crescentic glomerulonephritis |
publisher |
Elsevier |
series |
Kidney Medicine |
issn |
2590-0595 |
publishDate |
2021-07-01 |
description |
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. |
topic |
AL amyloidosis kidney amyloidosis rapidly progressive glomerulonephritis glomerular disease kidney biopsy |
url |
http://www.sciencedirect.com/science/article/pii/S2590059521000741 |
work_keys_str_mv |
AT annawang alamyloidosispresentingwithcrescenticglomerulonephritis AT yashpalskanwar alamyloidosispresentingwithcrescenticglomerulonephritis AT vikramaggarwal alamyloidosispresentingwithcrescenticglomerulonephritis AT anandsrivastava alamyloidosispresentingwithcrescenticglomerulonephritis |
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