Discrepancies between guidelines and international practice in treatment of hereditary angioedema.

Hereditary angioedema (HAE) is an autosomal dominant disorder characterized by decreased expression or loss of function of C1 esterase inhibitor (C1-INH). In 2010, international guidelines were published regarding the management of both acute HAE attacks and prophylactic treatment. Additionally, several clinical trials for HAE therapies were published in 2010. The purpose of this study was to assess the adherence of internationally based physicians to the current evidence-based studies and the 2010 International Consensus Algorithm. Internationally based physician members of the World Allergy Organization were surveyed regarding their diagnosis and management of patients with HAE. Only physicians who treat HAE patients were included in the analyses. Of the 201 responding physicians, the most highly used therapies for acute HAE attacks were C1-INH (59%), fresh frozen plasma (FFP; 42%), and icatibant (32%). For their preferred long-term prophylactic therapy, 74% used attenuated androgens and 18% used antifibrinolytics. Physicians in Latin and South America, in particular, were less likely than their international counterparts to prescribe C1-INH and more likely to prescribe attenuated androgens and FFP, and European physicians were the most likely to prescribe icatibant. Over one-third of physicians described themselves as "unfamiliar" with emerging HAE therapies. Many international physicians neither follow current evidence-based studies nor adhere to the 2010 International Consensus Algorithm for treating HAE. Further education of physicians, government authorities, and professional medical groups is necessary to enhance availability to therapies and appropriate use of them.