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What is HAE

Hereditary Angioedema (HAE) is a rare genetic condition which occurs in approximately 1 in 30,000 people worldwide. Symptoms include episodes of swelling in the hands, feet, abdomen, face and airway. Patients may suffer bouts of excruciating abdominal pain, nausea and vomiting that is exacerbated by swelling in the intestinal wall. Airway (laryngeal) swelling is particularly dangerous and can lead to death by asphyxiation. Untreated, attacks can last between 48 and 120 hours and can be fatal. Additional information about the condition can be found on the international HAE patient’s association website at

Medical need

C1 esterase inhibitor (C1INH) is a protein that naturally occurs in the human body. It belongs to the class of serine-protease inhibitors or serpins. It regulates several inflammatory pathways in the body by inhibiting certain proteins (proteases) that are part of the human immune system. Deficiency of functional C1 inhibitor leads to excessive activation of the complement system and other immunological and haemostatic pathways, giving cause to angioedema attacks. These attacks are characterized by acute and painful swellings of soft tissues. Administration of C1 inhibitor protein can normalize the low C1 INH levels and stop the angioedema attacks.


Pharming’s therapy

Patients who suffer from hereditary angioedema (HAE) have a deficiency of functional C1INH resulting from a mutation in the C1 inhibitor gene. For them, Pharming’s RUCONEST® provides a causal treatment with an excellent efficacy and safety profile. The GMP conform proprietary production and purification technology allows for the commercial manufacturing of large quantities of high-grade product of outstanding quality. RUCONEST® does not carry the risk of transmission of plasma related human infectious agents, as it is not isolated from human blood sources.


RUCONEST® is commercialized by Pharming in Algeria, Andorra, Austria, Bahrain, Belgium, France, Germany, Ireland, Jordan, Kuwait, Lebanon, Luxembourg, Morocco, the Netherlands, Oman, Portugal, Qatar, Syria, Spain, Switzerland, Tunisia, the United Arab Emirates, the United Kingdom, the United States of America and Yemen.

RUCONEST® is distributed by Swedish Orphan Biovitrum AB (publ) (SS: SOBI) in the other EU countries, and in Azerbaijan, Belarus, Georgia, Iceland, Kazakhstan, Liechtenstein, Norway, Russia, Serbia and Ukraine.

RUCONEST® is distributed in Argentina, Colombia, Costa Rica, the Dominican Republic, Panama, and Venezuela by Cytobioteck, in South Korea by HyupJin Corporation and in Israel by Kamada.

Distribution of Ruconest globally
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