[Profhylaxis and treatment of hereditary and acquired angioedema at HUB; use of the C1-esterase inhibitor]

Abstract

Introduction: Hereditary and acquired angioedema result from a deficiency in first complement component esterase (C1-esterase) inhibitor. It is characterized by transient subcutaneous tissue, intestinal wall, and upper airway swelling, which may lead to asphyxia and death. The pathophysiology, classification, diagnosis, and treatment of this condition are discussed, as is the follow-up of patients diagnosed with angioedema who received 1 vial of concentrated C1-INH (Berinert); the contribution of the Pharmacy Department in the dispensation and control of said drug is also assessed.

Material and methods: PubMed and other relevant sources were searched. Patient, dispensation, and Berinert(R) indication data were collected from controlled prescriptions; follow-up was completed by performing a medical history, collecting laboratory data, and patient interview when needed.

Results: Eight out of 9 cases were of hereditary angioedema, and 1 case was of acquired angioedema. Danazol was used as long-term prophylaxis in 8 patients, but side effects forced discontinuation in some of them. The C1-INH concentrate was effective in 6 acute episodes that were detected during the study, as well as in 3 surgical prophylaxis cases (overall use 6,500 U).

Conclusion: Danazol was effective as prophylactic treatment, but had severe side effects. The C1-INH concentrate was effective both as prophylactic therapy and treatment for acute episodes. An adequate stock of this product will be available at the Pharmacy Department.