Lisinopril-Induced Angioedema in a Patient with Plasma Prekallikrein DeficiencyFunding This study was supported in part by a grant from the Department of Veterans Affairs, Veterans Health Administration, Office of Research and Development, Biomedical Laboratory Research and Development (to P.T.), a grant from National Institutes of Health (HL139501 to P.T.). The contents of this manuscript are solely the responsibility of the authors and do not necessarily represent the views of the Department of Veterans Affairs or the United States Government.
26 August 2019
20 December 2019
23 January 2020 (online)
Angiotensin-converting enzyme (ACE) inhibitors are extensively prescribed to treat patients with hypertension, congestive heart failure, and diabetic nephropathy. A small fraction of these patients (approximately 0.7%) develop angioedema, manifested by swelling of the lips and oropharynx. Angioedema of oropharynx is a medical emergency that can lead to asphyxiation and death. The angioedema is due to bradykinin generated from high molecular weight kininogen by kallikrein, which is derived from plasma prekallikrein by action of the factor XIIa, factor Xia, or prolylcarboxypeptidase. Bradykinin induces vasodilation and increased vascular permeability. ACE is the major degrading enzyme of bradykinin in the intravascular department. ACE inhibitors inhibit the proteolytic inactivation of bradykinin. We report a patient with oropharyngeal angioedema associated with an ACE inhibitor with complete absence of plasma prekallikrein due to homozygous mutation (Ser97PhefsTer173).
P.T. analyzed and interpreted the data and wrote the manuscript. S.K.D. and S.R. performed the sequencing.
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