Treatment of hereditary angioedema: advances and gaps in care
摘要
Hereditary angioedema (HAE) is a rare genetic disorder that can cause sudden and repeated swelling in various parts of the body. In the respiratory tract, these attacks are potentially life-threatening. Since neither the timing of the attacks nor their location or severity can be predicted, the quality of life of affected patients is often severely impaired. Important therapeutic advances have been made in recent years and decades. For example, the possibility of self-administering medication has enabled those affected to respond quickly to HAE attacks. Another milestone was the establishment of long-term prophylaxis, which can guarantee freedom from attacks over a long period of time for some patients. Despite these advances, all affected individuals are required to always carry medication for acute treatment. The patient population is very heterogeneous and always requires individualized therapy planning in each case. It is important to adapt the management options to the respective life situations, which can be done at any time. In practice, the implementation of the therapy concept can be associated with various challenges. These will be discussed in this publication based on available data and our own experience to highlight the problems faced by patients and practitioners and how these barriers can potentially be removed or overcome.