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Disease Severity, Activity, Impact, and Control and How to Assess Them in Patients with Hereditary Angioedema
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Disease Severity, Activity, Impact, and Control and How to Assess Them in Patients with Hereditary Angioedema
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Journal Article
Disease Severity, Activity, Impact, and Control and How to Assess Them in Patients with Hereditary Angioedema
2017
Overview
Hereditary angioedema (HAE) is a group of rare, potentially life-threatening, and frequently debilitating diseases characterized by recurrent, and often with an unpredictable onset, of swelling attacks. HAE is heterogeneous, with considerable differences between its subtypes, patients, and even within the same patient over time. During the past few years, several new on demand and prophylactic therapies have become available for HAE, allowing for individualized treatment. Therefore, to optimize HAE management, it is important to determine in all patients, the severity of their attacks, their disease activity, its therapeutic control, and its impact on their quality of life. In this manuscript, we review the existing tools to assess these aspects of HAE management, many of which are patient-reported outcome instruments. Also, we outline the current gaps of knowledge and what tools are still missing to allow for a comprehensive assessment of all patients with HAE including children.
