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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Main Authors: 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
Format: Article
Language:English
Published: SAGE Publishing 2020-03-01
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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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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