Acquired Angioedema Due to C1-Inhibitor Deficiency (AAE-C1-INH)—A Bicenter Retrospective Study on Diagnosis, Course, and Therapy

Abstract

AAE-C1-INH is characterized by late-onset swellings mainly involving the face and low C1-INH levels. Diagnostic delay for AAE-C1-INH is further decreasing despite being about 10-fold rarer than HAE. Patients severely affected without underlying condition or no indication for treatment could benefit from off-label therapy.