Biotech company Pharming Group NV (Pharming) announced that they have agreed an extension of their existing agreement with Swedish Orphan Biovitrum (SOBI) to include new territories in the Balkans, North Africa and the Middle East for the commercialization of Ruconest (recombinant human C1 inhibitor) for the treatment of acute angioedema attacks in patients with Hereditary Angioedema (HAE).

In addition, SOBI has placed a significant additional order for vials of Ruconest. The shipment forms part of SOBI’s preparation for the continued roll-out and intensified marketing of Ruconest across Europe. SOBI will over a period of 1 year pay €1.5 million to Pharming for these additional shipments under this order. Under the terms of the distribution partnership, SOBI buys finished product from Pharming for a transfer price that includes a sales related tiered royalty component.

Sijmen de Vries, CEO of Pharming, said “We are delighted to expand our current commercialization deal with SOBI. This is another significant step towards making Ruconest more widely available. With this extension, patients in all EC territories, the Balkans, North Africa and the Middle East will soon be able to benefit from the efficacy and safety that Ruconest offers as the only recombinant enzyme replacement therapy for HAE.”

Ruconest / Rhucin (INN conestat alfa) is a recombinant version of the human protein C1 inhibitor (C1INH). Rhucin is produced through Pharming’s proprietary technology in milk of transgenic rabbits and in Europe is approved under the name Ruconest for treatment of acute angioedema attacks in patients with HAE. Rhucin has been granted orphan drug designation in the US for the treatment of acute attacks of HAE, a genetic disorder in which the patient is deficient in or lacks a functional plasma protein C1 inhibitor, resulting in unpredictable and debilitating episodes of intense swelling of the extremities, face, trunk, genitals, abdomen and upper airway. The frequency and severity of HAE attacks vary and are most serious when they involve laryngeal edema, which can close the upper airway and cause death by asphyxiation. According to the US Hereditary Angioedema Association, epidemiological estimates for HAE range from one in 10,000 to one in 50,000 individuals.

Pharming Group NV is developing innovative products for the treatment of unmet medical needs. Ruconest (Rhucin in non-European territories) is a recombinant human C1 inhibitor approved for the treatment of angioedema attacks in patients with HAE in all 27 EU countries plus Norway, Iceland and Liechtenstein, and is distributed in the EU by SOBI.