Pharming Group Confirms Receipt Of US$20 Million Milestone Payment From Salix Pharmaceuticals, Ltd.

Leiden, 04 November 2014 - Biotech company Pharming Group NV (“Pharming” or “the Company”) (EURONEXT: PHARM), today announced the receipt of a US$20 million milestone payment from Salix Pharmaceuticals (“Salix”). The milestone was paid according to the terms and conditions of the Ruconest® commercialization agreement between Salix and Pharming.

Sijmen de Vries, Pharming CEO, commented, “The US launch of Ruconest®, as announced yesterday, and the receipt of the US$20 million milestone from Salix, mark what we perceive as the beginning of a new era for Pharming. The receipt of this milestone payment strengthens our debt-free balance sheet to more than €38 million as of today. From this solid basis, as result of future receipts of 30% of US net sales, up to $100 million annual sales, increasing stepwise up to 40% for annual US sales in excess of US$100 million for the supply of Ruconest® to Salix and in addition growing revenues from EU sales, both from our own direct commercialization and by Sobi, we are now aiming for our goal of achieving financial sustainability of the Company.

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About Pharming Group NV:

Pharming Group NV is developing innovative products for the treatment of unmet medical needs. Ruconest® (conestat alfa) is a recombinant human C1 esterase inhibitor approved for the treatment of angioedema attacks in patients with HAE in the USA, Israel, all 27 EU countries plus Norway, Iceland and Liechtenstein. Ruconest is commercialized by Pharming in Austria, Germany and Netherlands. 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 partnered with Salix Pharmaceuticals Inc. (NASDAQ: SLXP) in North America.

Ruconest is also being investigated in a randomized Phase II clinical trial for prophylaxis of HAE and evaluated for various additional follow-on indications. Pharming has a unique GMP compliant, validated platform for the production of recombinant human proteins that has proven capable of producing industrial volumes of high quality recombinant human protein in a more economical way compared to current cell based technologies. Leads for enzyme replacement therapy in Pompe’s, Fabry’s and Gaucher’s diseases are under early evaluation. The platform is partnered with Shanghai Institute for Pharmaceutical Industry (SIPI), a Sinopharm Company, for joint global development of new products. Pre- clinical development and manufacturing will take place at SIPI and are funded by SIPI. Pharming and SIPI initially plan to utilize this platform for the development of rh-FVIII for the treatment of Haemophilia-A. Additional information is available on the Pharming website; www.pharming.com.

This press release contains forward looking statements that involve known and unknown risks, uncertainties and other factors, which may cause the actual results, performance or achievements of the Company to be materially different from the results, performance or achievements expressed or implied by these forward looking statements.

Contact

Sijmen de Vries, CEO: T: +31 71 524 7400
FTI Consulting
Julia Phillips/ Victoria Foster Mitchell, T: +44 203 727 1136

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