Lupus-associated Angioedema Successfully Treated with C1 Esterase Inhibitor Concentrate in an Adolescent Female: A Case Report

Abstract

Introduction: Acquired angioedema is a rare complication of systemic lupus erythematosus (SLE). This type of angioedema is bradykinin-mediated and thought to be due to an acquired antibody against C1 esterase inhibitor (C1-INH). It can be difficult to manage and is often refractory to standard therapies. This case aims to describe the presentation of refractory angioedema as a presenting feature of SLE in a patient and her clinical course. She was treated with intravenous C1-INH and had a dramatic clinical improvement. The use of intravenous C1-INH in lupus-associated angioedema has …