| Autor: |
Brendan L. Thoms, Varun Agrawal, Elvira R. Umyarova, Pamela C. Gibson, Richard J. Solomon |
| Jazyk: |
angličtina |
| Rok vydání: |
2021 |
| Předmět: |
|
| Zdroj: |
Case Reports in Nephrology and Dialysis, Vol 11, Iss 2, Pp 183-189 (2021) |
| Druh dokumentu: |
article |
| ISSN: |
2296-9705 |
| DOI: |
10.1159/000517142 |
| Popis: |
Antineutrophil cytoplasmic autoantibody (ANCA) vasculitis has occasionally been associated with other systemic glomerulonephritis, such as anti-glomerular basement membrane disease. Here, we report the first clinical case of ANCA-associated crescentic glomerulonephritis with AL amyloidosis. An 81-years-old gentleman presented to the hospital with acute kidney injury (serum creatinine 4.7 mg/dL) on a background of chronic kidney disease and volume overload. Autoimmune serology was remarkable for p-ANCA and myeloperoxidase positivity. A renal biopsy confirmed pauci-immune glomerulonephritis and lambda light-chain amyloid deposition (confirmed on liquid chromatography and tandem mass spectrometry). The patient was initially managed with rituximab and subsequently transitioned to bortezomib-based chemotherapy but died due to decompensated heart failure. This case report promotes greater awareness of the unusual presentation of amyloidosis and guides future research and treatment. |
| Databáze: |
Directory of Open Access Journals |
| Externí odkaz: |
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