Pharming Group NV (EURONEXT: PHARM) and Salix Pharmaceuticals, Ltd. (NASDAQ:SLXP)
today announced that the first patient was treated in their Phase 2 clinical study of
Ruconest(R), (C1 Esterase Inhibitor [Recombinant]) 50 IU/kg, for prophylaxis in patients
with hereditary angioedema (HAE).

    HAE patients deficient in C1 inhibitor and with a history of at least four attacks per
month are being enrolled in the randomized, double-blind study, in which 30 patients will
receive Ruconest(R) either once or twice weekly, or placebo in each of 3 treatment
periods. With the crossover design, all patients will receive each of the dosing regimens.
The study will evaluate the safety and efficacy of Ruconest(R) when used for prophylaxis
of angioedema attacks in patients with HAE.

    The study will be conducted at sites in Canada, Europe, Israel, and the United States.
The trial is being coordinated by principal investigators, Dr. Marco Cicardi, Professor at
the University of Milan, and Dr. Marc Riedl, Associate Professor and Clinical Director of
the US HAEA Angioedema Center at the University of California, San Diego.

    "We are pleased to have quickly initiated the treatment phase of this important study,
and look forward to its completion later in 2015," said Bruno Giannetti, MD PhD, Chief
Operating Officer of Pharming.

    Under the terms of the Pharming-Salix license agreement, the companies will equally
share the development costs for Ruconest(R) for HAE prophylaxis. Pharming will receive an
undisclosed milestone payment from Salix as and when FDA approval for this additional
indication is given.

    Important Safety Information for RUCONEST

    Indication:

    RUCONEST(R) is a C1 esterase inhibitor [recombinant] indicated for the treatment of
acute attacks in adult and adolescent patients with hereditary angioedema (HAE).
Effectiveness in clinical studies was not established in HAE patients with laryngeal
attacks.

    RUCONEST(R) (C1 esterase inhibitor [recombinant]) is contraindicated in patients with
a history of allergy to rabbits or rabbit-derived products, and patients with a history of
life-threatening immediate hypersensitivity reactions to C1 esterase inhibitor
preparations, including anaphylaxis.

    Severe hypersensitivity reactions may occur. The signs and symptoms of
hypersensitivity reactions may include hives, generalized urticaria, tightness of the
chest, wheezing, hypotension, and/or anaphylaxis during or after injection of RUCONEST.
Should symptoms occur, discontinue RUCONEST and administer appropriate treatment. Because
hypersensitivity reactions may have symptoms similar to HAE attacks, treatment methods
should be carefully considered.

    Serious arterial and venous thromboembolic (TE) events have been reported at the
recommended dose of plasma derived C1 esterase inhibitor products in patients with risk
factors. Risk factors may include the presence of an indwelling venous catheter/access
device, prior history of thrombosis, underlying atherosclerosis, use of oral
contraceptives or certain androgens, morbid obesity, and immobility. Monitor patients with
known risk factors for TE events during and after RUCONEST administration.

    RUCONEST has not been studied in pregnant women; therefore, it should only be used
during pregnancy if clearly needed.

    The most common adverse reactions (incidence greater than or equal to2%) were
headache, nausea, and diarrhea. The serious adverse reaction in clinical studies of
RUCONEST was anaphylaxis.

    Please see complete Prescribing Information for RUCONEST.

    About RUCONEST(R)

    RUCONEST(R) (C1 Esterase Inhibitor [Recombinant]) 50 IU/kg is an injectable medicine
that is used to treat acute angioedema attacks in adult and adolescent patients with
hereditary angioedema (HAE). HAE is caused by a deficiency of the C1 esterase inhibitor
protein, which is present in blood and helps control inflammation (swelling) and parts of
the immune system. A shortage of C1 esterase inhibitor can lead to repeated attacks of
swelling, pain in the abdomen, difficulty breathing and other symptoms. RUCONEST(R)
contains C1 esterase inhibitor at 50 IU/kg.

    When administered at the onset of HAE attack symptoms at the recommended dose,
RUCONEST works to return a patient's C1-INH levels to normal range and quickly begins to
relieve the symptoms of an HAE attack with a low recurrence of symptoms.

    RUCONEST is the first and only plasma-free, recombinant C1-INH approval from the U.S.
Food and Drug Administration (FDA) and was approved in July 2014.

    RUCONEST has been granted Orphan Drug designation by the FDA for the treatment of
acute angioedema attacks in patients with hereditary angioedema (HAE). With RUCONESTnow
approved by the FDA, Salix believes this designation should provide seven years of
marketing exclusivity in the United States.

    About HAE

    Hereditary angioedema (HAE) is a genetic condition occurring between 1 in 10,000 to 1
in 50,000 people. Those with HAE experience episodes of swelling in their extremities,
face and abdomen, with potentially life-threatening swelling of the airway. When it occurs
in the abdomen, this swelling can be accompanied by bouts of nausea, vomiting and severe
pain. Swelling in the face or extremities can be painful, disfiguring, and disabling.

    HAE patients have a defect in the gene that controls production of a protein found in
the blood vessels, called C1 inhibitor or C1-INH. When a person's C1-INH levels are low,
fluid from blood vessels can leak into nearby connective tissues, causing severe pain and
swelling and, in rare cases, death from asphyxiation from airway swelling.

    About Pharming Group NV

    Pharming Group NV is developing innovative products for the treatment of unmet medical
needs. Ruconest(R) (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; http://www.pharming.com.

    About Salix Pharmaceuticals

    Salix Pharmaceuticals, Ltd., headquartered in Raleigh, North Carolina, develops and
markets prescription pharmaceutical products and medical devices for the prevention and
treatment of gastrointestinal diseases. Salix's strategy is to in-license late-stage or
marketed proprietary therapeutic products, complete any required development and
regulatory submission of these products, and commercialize them through the Company's
500-member specialty sales force.

    Salix trades on the NASDAQ Global Select Market under the ticker symbol "SLXP". For
more information, please visit our website at http://www.salix.com
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or contact Salix at 919-862-1000. Follow us on Twitter (@SalixPharma)
and Facebook (http://www.facebook.com/SalixPharma
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). Information on our Twitter feed,
Facebook page and website is not incorporated in our filings with the SEC.

    Salix Disclosure Notice

    Please Note: The statements provided herein that are not historical facts are or might
constitute projections and other forward-looking statements regarding future events.
Although we believe the expectations reflected in such forward-looking statements are
based on reasonable assumptions, our expectations might not be attained. Forward-looking
statements are just predictions and are subject to known and unknown risks and
uncertainties that could cause actual events or results to differ materially from expected
results. Factors that could cause actual events or results to differ materially from those
described herein include, among others: uncertainty that Ruconest will be commercially
successful; market acceptance for approved products; generic and other competition in an
increasingly global industry; litigation and the possible impairment of, or inability to
obtain, intellectual property rights and the costs of obtaining such rights from third
parties in an increasingly global industry; the unpredictability of the duration and
results of regulatory review of New Drug Applications, Biologics License Agreements and
Investigational NDAs, including risk that XIFAXAN (rifaximin) 550 mg will not receive the
necessary regulatory approvals for Irritable Bowel Syndrome with Diarrhea; the cost,
timing and results of clinical trials and other development activities involving
pharmaceutical products; post-marketing approval regulation, including the ongoing
Department of Justice investigation of Salix's marketing practices; revenue recognition
and other critical accounting policies; the need to acquire new products; changes in tax
laws or interpretations thereof; general economic and business conditions; and other
factors. Readers are cautioned not to place undue reliance on the forward-looking
statements included herein, which speak only as of the date hereof. Salix does not
undertake to update any of these statements in light of new information or future events,
except as required by law. The reader is referred to the documents that Salix files from
time to time with the SEC.

    Pharming Disclosure Notice

    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.


    Contacts:

    Pharming Group NV

    Sijmen de Vries
    Chief Executive Officer
    +31-71-5247400

    Julia Phillips/ Victoria Foster Mitchell
    FTI Consulting
    +44-203-727-1136

    Salix Pharmaceuticals

    Timothy J. Creech
    Senior Vice President
    Finance & Administrative Services and Acting Chief Financial Officer
    +1-919-862-1000

    G. Michael Freeman
    Associate Vice President, Investor Relations and Corporate Communications
    +1-919-862-1000


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