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An Unusual Case of Acquired Angioedema and Monoclonal Gammopathy of Renal Significance in a Middle-Aged Caucasian Female #MMPMID32167383
Roy S; Konala VM; Kyaw T; Chakraborty S; Naramala S; Gayam V; Adapa S; Bose S
J Investig Med High Impact Case Rep 2020[Jan]; 8 (?): 2324709620912096 PMID32167383show ga
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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J+Investig+Med+High+Impact+Case+Rep 2020 ; 8 (?): 2324709620912096
