Affiliation:
1. From the Division of Immunology and Allergy, Department of Pediatrics, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio;
2. Division of Immunology/Allergy Section, Department of Internal Medicine, University of Cincinnati College of Medicine, Cincinnati, Ohio; and
Abstract
Background: Acquired angioedema (AAE) is a rare form of angioedema (AE) and is often associated with lymphoproliferative conditions and/or anti‐C1 esterase inhibitor (C1-INH) antibodies without clear treatment consensus. Current treatments have been reported to have
variable effectiveness with different safety concerns. A large Italian cohort of patients with AAE was previously found to respond well to tranexamic acid (TXA). Herein, we report our experience treating AAE with TXA used as prophylaxis. Objective: The objective was to
describe clinical characteristics of patients with AAE and to report our experience with treating AAE with TXA. Methods: A retrospective chart review of patients with AAE (N = 13) from a large practice was conducted to assess characteristics and treatment responses. Patient
demographics in addition to C1-INH quantitative, C1-INH functional, C4, and C1q levels; the presence of C1-INH antibodies; and a history of lymphoproliferative disease were extracted. The patients were also characterized by their treatment response to TXA. Results: All
the patients were white, with a mean age at diagnosis of 67 years, an average body mass index of 31.3 kg/m2, and a male-to-female ratio of 7:6. Nine patients had positive C1-INH antibodies. The patients were on various prophylaxis treatments before TXA, including chemotherapy that
targeted malignancy, cyclophosphamide, rituximab, and plasmapheresis. Ultimately, 11 of the 13 patients were on TXA for prophylaxis. At 1, 12, and 24 months after TXA treatment, attacks decreased by 97, 86, and 99%, respectively. One patient developed a deep vein thrombosis and TXA was stopped.
Conclusion: These findings demonstrated that treatment of AAE with TXA was effective as prophylaxis for AE attacks. However, potential adverse effects remain a concern, which emphasizes the need for additional options.
Publisher
Oceanside Publications Inc.
Subject
Pulmonary and Respiratory Medicine,General Medicine,Immunology and Allergy
Cited by
5 articles.
订阅此论文施引文献
订阅此论文施引文献,注册后可以免费订阅5篇论文的施引文献,订阅后可以查看论文全部施引文献