An Unusual Case of Acquired Angioedema and Monoclonal Gammopathy of Renal Significance in a Middle-Aged Caucasian Female
Acquired angioedema due to deficiency of C1 esterase inhibitor is also called acquired angioedema and is abbreviated as C1INH-AAE. It is a rare syndrome of recurrent episodes of angioedema, without urticaria, and in some patients, it is associated with B-cell lymphoproliferative disorders. Kidney in...
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SAGE Publishing
2020-03-01
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Series: | Journal of Investigative Medicine High Impact Case Reports |
Online Access: | https://doi.org/10.1177/2324709620912096 |
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author | Sasmit Roy MD Venu Madhav Konala MD Thurein Kyaw MD Sandipan Chakraborty MD Srikanth Naramala MD Vijay Gayam MD Sreedhar Adapa MD Subhasish Bose MD |
author_facet | Sasmit Roy MD Venu Madhav Konala MD Thurein Kyaw MD Sandipan Chakraborty MD Srikanth Naramala MD Vijay Gayam MD Sreedhar Adapa MD Subhasish Bose MD |
author_sort | Sasmit Roy MD |
collection | DOAJ |
description | Acquired angioedema due to deficiency of C1 esterase inhibitor is also called acquired angioedema and is abbreviated as C1INH-AAE. It is a rare syndrome of recurrent episodes of angioedema, without urticaria, and in some patients, it is associated with B-cell lymphoproliferative disorders. Kidney involvement is rare in this condition. The monoclonal immunoglobulin secreted by a nonmalignant or premalignant B-cell or plasma cell clone, causing renal damage that represents a group of disorders which are termed as monoclonal gammopathy of renal significance (MGRS). In this article, we report a rare case of acquired C1 esterase deficiency angioedema and acute kidney injury with renal biopsy-proven MGRS. We present a 64-year-old Caucasian woman who presented with 2 weeks of recurring urticaria and new onset of acute kidney injury. She was diagnosed with monoclonal gammopathy–associated proliferative glomerulopathy through kidney biopsy, and serological workup came back positive for C1 esterase deficiency, implying acquired angioedema. Acquired angioedema is a rare disease with systemic involvement. Recurrent allergic manifestations and acute kidney injury should prompt MGRS as a differential. |
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issn | 2324-7096 |
language | English |
last_indexed | 2024-12-23T04:43:28Z |
publishDate | 2020-03-01 |
publisher | SAGE Publishing |
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series | Journal of Investigative Medicine High Impact Case Reports |
spelling | doaj.art-140279e131a3460a88eb2398b8689d7e2022-12-21T17:59:43ZengSAGE PublishingJournal of Investigative Medicine High Impact Case Reports2324-70962020-03-01810.1177/2324709620912096An Unusual Case of Acquired Angioedema and Monoclonal Gammopathy of Renal Significance in a Middle-Aged Caucasian FemaleSasmit Roy MD0Venu Madhav Konala MD1Thurein Kyaw MD2Sandipan Chakraborty MD3Srikanth Naramala MD4Vijay Gayam MD5Sreedhar Adapa MD6Subhasish Bose MD7Lynchburg Nephrology Physicians, Lynchburg, VA, USAAshland Bellefonte Cancer Center, Ashland, KY, USALynchburg Nephrology Physicians, Lynchburg, VA, USAMiami Valley Hospital, Dayton, OH, USAAdventist Medical Center, Hanford, CA, USAInterfaith Medical Center, Brooklyn, NY, USAThe Nephrology Group, Fresno, CA, USALynchburg Nephrology Physicians, Lynchburg, VA, USAAcquired angioedema due to deficiency of C1 esterase inhibitor is also called acquired angioedema and is abbreviated as C1INH-AAE. It is a rare syndrome of recurrent episodes of angioedema, without urticaria, and in some patients, it is associated with B-cell lymphoproliferative disorders. Kidney involvement is rare in this condition. The monoclonal immunoglobulin secreted by a nonmalignant or premalignant B-cell or plasma cell clone, causing renal damage that represents a group of disorders which are termed as monoclonal gammopathy of renal significance (MGRS). In this article, we report a rare case of acquired C1 esterase deficiency angioedema and acute kidney injury with renal biopsy-proven MGRS. We present a 64-year-old Caucasian woman who presented with 2 weeks of recurring urticaria and new onset of acute kidney injury. She was diagnosed with monoclonal gammopathy–associated proliferative glomerulopathy through kidney biopsy, and serological workup came back positive for C1 esterase deficiency, implying acquired angioedema. Acquired angioedema is a rare disease with systemic involvement. Recurrent allergic manifestations and acute kidney injury should prompt MGRS as a differential.https://doi.org/10.1177/2324709620912096 |
spellingShingle | Sasmit Roy MD Venu Madhav Konala MD Thurein Kyaw MD Sandipan Chakraborty MD Srikanth Naramala MD Vijay Gayam MD Sreedhar Adapa MD Subhasish Bose MD An Unusual Case of Acquired Angioedema and Monoclonal Gammopathy of Renal Significance in a Middle-Aged Caucasian Female Journal of Investigative Medicine High Impact Case Reports |
title | An Unusual Case of Acquired Angioedema and Monoclonal Gammopathy of Renal Significance in a Middle-Aged Caucasian Female |
title_full | An Unusual Case of Acquired Angioedema and Monoclonal Gammopathy of Renal Significance in a Middle-Aged Caucasian Female |
title_fullStr | An Unusual Case of Acquired Angioedema and Monoclonal Gammopathy of Renal Significance in a Middle-Aged Caucasian Female |
title_full_unstemmed | An Unusual Case of Acquired Angioedema and Monoclonal Gammopathy of Renal Significance in a Middle-Aged Caucasian Female |
title_short | An Unusual Case of Acquired Angioedema and Monoclonal Gammopathy of Renal Significance in a Middle-Aged Caucasian Female |
title_sort | unusual case of acquired angioedema and monoclonal gammopathy of renal significance in a middle aged caucasian female |
url | https://doi.org/10.1177/2324709620912096 |
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