Clinical manifestations of hereditary angioedema: a systematic review of treatment

Hereditary Angioedema or HAE is a rare autosomal dominant disease leading to three major types of acute swelling and is known to be triggered by C1 esterase inhibitor deficiency. The purpose of this systematic review was to summarize the key clinical features of HAE and to compare and analyze the available management options and potential new management prospects. For this study, their evaluation of cases showed that 45% of our HAE patients present with peripheral edema and 30% with abdominal pain, 15% with facial swelling, and 10% with larynx edema. The review of the treatment’s effectiveness showed that C1-INH replacement therapy forms the mainstay of HAE management and proved to deliver great success both, in acute attack management and long-term prevention. H2 Subcutaneous Bradykinin receptor antagonists were also established as an effective form of treatment which is quite reliable and more acceptable by the patients. The study evidences suggest that newer interferences, such as monoclonal antibodies and gene therapy, may hold significant potential in the course of HAE treatment, although additional investigations should provide definitive information related to the safety and efficacy in the long-term management of disease. The use of technologies in smart monitoring for health, and Integrated Digital Health System has become important tool to detect early attacks and for better results. In detailing the results, differences in treatment strategies also highlight how HAE attacks are difficult to predict and how clinical manifestations of the disease could be different from one patient to the other. The review also pointed out prominent limitations of current research, such as the comparison of the effectiveness between the newer treatments and the costs of administering these new treatments. Collectively, these conclusions support the notion that much advancement has taken place in the management of HAE and yet there remains much potential for optimizing the HAE patient’s care by development of better diagnostic, treatment and patient-completed and integration of the corresponding advanced technologies. Future concerns should include further refinement of the costs of treatments, the continued fine-tuning of prophylaxis, and long-term safety assessment of the newer therapies. These findings further knowledge in the management of HAE and offer useful guidelines in the management of HAE for health professionals.