Hereditary Angioedema (HAE) is an autosomal dominant disease which is caused by a deficiency in functional C1 inhibitor. It is a rare hereditary disease which is characterized by painful, unpredictable, recurrent attacks of inflammation and swelling affecting the hands, feet, face, abdomen, urogenital tract, and the larynx. This genetic disease is caused by the production of either inadequate or nonfunctioning C1-Inhibitor protein. The C1-Inhibitor helps in the regulation of the complex biochemical interactions of blood-based systems which are involved in disease fighting, inflammatory responses and coagulation.
It is very rare and potentially life-threatening genetic disorder. Hereditary Angioedema is classified as Hereditary Angioedema Type I, Hereditary Angioedema Type II and Hereditary Angioedema Type III on the basis of causes and levels of the protein called C1 inhibitor in the blood. United State accounts for the highest number of prevalent cases of HAE followed by EU5 and Japan. DelveInsight estimates that the prevalence of HAE will increase significantly during the period of 2015-2025 in the 7MM. As per the records HAE type I is more frequent form as compared to HAE type II and HAE type III in the 7MM.
With the active involvement of big pharma companies HAE market is continuously growing. Shire is currently dominating the HAE market with the leading treatment for both: the long-term prophylaxis (Cinryze), and the acute treatment (Firazyr). Shire with the acquisition of Dyax in November 2015, got an access to their lead pipeline product, DX-2930, which will expand & extends Shire’s industry-leading HAE portfolio. Even after having a robust HAE drug pipeline the current treatments have limitations due to cost, side effects and access to the treatment. Some market drivers facilitating the HAE market growth are Investments in development and research, Emerging Therapies, and Socio-Economic Factors.