The New England Journal of Medicine Publishes Pivotal Data Demonstrating Prophylactic Infusions of ALPROLIX™ Effectively

  The New England Journal of Medicine Publishes Pivotal Data Demonstrating
  Prophylactic Infusions of ALPROLIX™ Effectively Controlled Bleeding in
  Hemophilia B Patients

 – B-LONG Study Showed Investigational Therapy Administered Once Every One to
              Two Weeks Prevented or Reduced Bleeding Episodes –

Business Wire

CAMBRIDGE, Mass. & STOCKHOLM -- December 4, 2013

Today Biogen Idec (NASDAQ: BIIB) and Swedish Orphan Biovitrum AB (publ) (Sobi)
(STO: SOBI) announced the publication of detailed results from the pivotal
Phase 3 study of ALPROLIX^TM, the companies’ investigational long-lasting
recombinant factor IX Fc fusion protein candidate for hemophilia B. The study
appears in the Online First edition and will appear in the December 12 print
issue of The New England Journal of Medicine (NEJM).

The study of ALPROLIX showed that people with severe hemophilia B safely and
effectively prevented or reduced bleeding episodes with prophylactic infusions
every one to two weeks. As the first long-lasting investigational therapy for
hemophilia B to complete a Phase 3 study, ALPROLIX has the potential to be the
first important advance in hemophilia B treatment in more than 16 years.

The study, called B-LONG, is the largest Phase 3 clinical study in hemophilia
B ever completed. It examined the effect of ALPROLIX therapy delivered with
multiple dosing regimens, including prophylactic (weekly or longer), episodic
(on-demand) and surgical (perioperative management). Results of B-LONG formed
the basis of regulatory applications for ALPROLIX, which are currently under
review in several countries including the United States, Canada, Australia and
Japan.

“Today, many people with hemophilia B do not follow a prophylactic regimen,
and the burdensome infusion schedules associated with currently available
treatment may contribute to its limited adoption,” said Marilyn Manco-Johnson,
M.D., professor of Pediatrics, University of Colorado and director, Hemophilia
and Thrombosis Center, University of Colorado and Children’s Hospital,
Colorado. “The National Hemophilia Foundation recommends a prophylactic
regimen as optimal for people with severe hemophilia, and studies show this
approach reduces bleeding episodes and associated risks. It is my hope that
long-lasting therapies in development, such as ALPROLIX, may lessen the burden
of prophylactic dosing for people with hemophilia B, and encourage adoption.”

Hemophilia B is a rare, chronic, inherited disorder in which the ability of a
person’s blood to clot is impaired, which can lead to extended bleeding
episodes. Currently available therapy requires prophylactic infusions two
times a week or more. Frequent prophylactic infusions can be a burden to
people with hemophilia and may reduce adoption to this type of treatment
regimen.^1 ALPROLIX has a prolonged circulation time in the blood, and
utilizes a technology called Fc fusion. The B-LONG study showed that the
interval between prophylactic infusions was extended with ALPROLIX, so that
infusions were only needed once a week to once every two weeks in the study.
If approved, ALPROLIX may enable people with severe hemophilia B to receive
fewer prophylactic infusions than currently available therapy.

“Many of us at Biogen Idec have personal connections to the hemophilia
community and know firsthand the burden of frequent infusions,” said Glenn
Pierce, M.D., Ph.D., senior vice president of Global Medical Affairs and chief
medical officer at Biogen Idec’s hemophilia therapeutic area. “If approved,
long-lasting ALPROLIX therapy will have the potential to change the way
hemophilia B is managed and address a critical need for patients.”

B-LONG Study Results
B-LONG was a global, open-label, multi-center Phase 3 study that evaluated the
efficacy, safety and pharmacokinetics (measurement of the presence of the drug
in a patient’s body over time) of ALPROLIX in 123 males aged 12 years and
older with hemophilia. The study involved 50 hemophilia treatment centers in
17 countries on six continents.

The B-LONG study evaluated ALPROLIX via four treatment regimens:

  *Weekly prophylaxis (arm 1)
  *Individualized-interval prophylaxis dosing – starting at every 10 days
    (arm 2)
  *Episodic (on-demand) therapy as needed to manage bleeding episodes (arm 3)
  *Perioperative (surgical) management (arm 4)

Dose (arm 1) and interval (arm 2) were adjusted during the study to maintain
target factor IX levels intended to prevent bleeding.

The overall median annualized bleeding rates (ABR), or projected rate of
bleeding episodes per year, were 3.0 for weekly prophylaxis arm and 1.4 for
individualized-interval prophylactic regimens, compared to 17.7 for the
episodic therapy group. The median dosing interval with
individualized-interval prophylaxis (arm 2) was 12.5 days. Bleeding episodes
for participants in arms 1-3 were documented and more than 90 percent of all
bleeding episodes were controlled by a single infusion of ALPROLIX. ALPROLIX
was assessed in the perioperative management of 12 study participants
undergoing 14 major surgical procedures. The treating physicians rated
response to surgery of ALPROLIX as “excellent” or “good” in 100 percent of
these surgeries.

Noparticipants in the study developed inhibitors to ALPROLIX (antibodies that
may interfere with the activity of the therapy). There were no reports of
vascular clots or serious allergic reactions. Overall, safety events were
consistent with those expected in the general hemophilia population. The most
common adverse events (incidence of ≥5 percent) occurring outside of the
perioperative period were nasopharyngitis (common cold), influenza (flu),
arthralgia (joint pain), upper respiratory infection, hypertension (high blood
pressure) and headache. One participant had a single serious adverse event
that was considered to be possibly related to treatment with ALPROLIX. The
participant, who had a history of hematuria (presence of blood in the urine),
developed an obstructive clot in the urinary collecting system; he continued
ALPROLIX treatment and the event resolved with medical management.

“The publication of the B-LONG pivotal study in The New England Journal of
Medicine is a significant milestone that will contribute to the advancement of
medical science in hemophilia care,” said Birgitte Volck, M.D., Ph.D., senior
vice president development and chief medical officer of Sobi. “These data
support the potential of ALPROLIX to provide a meaningful new option to people
with hemophilia B by addressing the need for long-lasting therapies for this
population.”

About ALPROLIX
ALPROLIX is an investigational fully recombinant, long-lasting clotting factor
therapy being developed for hemophilia B. It uses Fc fusion technology, which
takes advantage of a naturally occurring pathway that delays the breakdown of
Immunoglobulin G Subclass 1, or IgG1 (protein commonly found in the body), and
cycles it back into the bloodstream. The Fc portion of IgG1 is fused to factor
IX in ALPROLIX and is thought to be responsible for the prolonged time
ALPROLIX circulates in the body. While Fc fusion is an established technology
that has been used for more than 15 years, Biogen Idec is the only company to
apply it in hemophilia.

About Hemophilia B
Hemophilia B is a rare, inherited disorder in which the ability of a person’s
blood to clot is impaired. Hemophilia B occurs in about one in 25,000 male
births annually, and more rarely in females, affecting about 3,300 people in
the United States. The World Federation of Hemophilia global survey conducted
in 2011 estimates that more than 25,000 people are currently diagnosed with
hemophilia B worldwide. It is caused by having substantially reduced or no
factor IX activity, which is needed for normal blood clotting. People with
hemophilia B experience prolonged bleeding episodes that can cause pain,
irreversible joint damage and life-threatening hemorrhages. Prophylactic
infusions of factor IX can temporarily replace the missing clotting factors
that are needed to control bleeding and prevent new bleeding episodes. The
Medical and Scientific Advisory Council of the National Hemophilia Foundation
recommends prophylaxis as the optimal therapy for people with severe
hemophilia B.

About the Biogen Idec and Sobi Collaboration
Biogen Idec and Swedish Orphan Biovitrum (Sobi) are partners in the
development and commercialization of ALPROLIX for hemophilia B. Biogen Idec
leads development, has manufacturing rights, and has commercialization rights
in North America and all other regions excluding the Sobi territory. Sobi has
the right to opt in to assume final development and commercialization in
Europe (including Russia), the Middle East and Northern Africa.

AboutBiogen Idec
Through cutting-edge science and medicine,Biogen Idecdiscovers, develops and
delivers to patients worldwide innovative therapies for the treatment of
neurodegenerative diseases, hemophilia and autoimmune disorders. Founded in
1978,Biogen Idecis the world’s oldest independent biotechnology company.
Patients worldwide benefit from its leading multiple sclerosis therapies, and
the company generates more than$5 billionin annual revenues. For product
labeling, press releases and additional information about the company, please
visit www.biogenidec.com.

About Sobi
Sobi is an international specialty healthcare company dedicated to rare
diseases. Our mission is to develop and deliver innovative therapies and
services to improve the lives of patients. The product portfolio is primarily
focused on inflammation and genetic diseases, with three late stage biological
development projects within hemophilia and neonatology. We also market a
portfolio of specialty and rare disease products for partner companies. Sobi
is a pioneer in biotechnology with world-class capabilities in protein
biochemistry and biologics manufacturing. In 2012, Sobi had total revenues of
SEK 1.9 billion (€ 215 M) and about 500 employees. The share (STO: SOBI) is
listed on NASDAQ OMX Stockholm. More information is available at www.sobi.com.

Biogen Idec Safe Harbor
This press release contains forward-looking statements, including statements
about the potential advances, impact and therapeutic effect of ALPROLIX, our
investigational long-lasting recombinant factor IX candidate. These statements
may be identified by words such as "believe," "expect," "may," "plan,"
"potential," "will" and similar expressions, and are based on our current
beliefs and expectations. Drug development and commercialization involve a
high degree of risk. Factors which could cause actual results to differ
materially from our current expectations include the risk that unexpected
concerns may arise from additional data or analysis, regulatory authorities
may require additional data or information or further studies, or may fail to
approve or may delay approval of our drug candidates, or we may encounter
other unexpected hurdles. For more detailed information on the risks and
uncertainties associated with our drug development and commercialization
activities, please review the Risk Factors section of our most recent annual
or quarterly report filed with the Securities and Exchange Commission. Any
forward-looking statements speak only as of the date of this press release and
we assume no obligation to update any forward-looking statements, whether as a
result of new information, future events or otherwise.

^1 Hacker MR, Geraghty S, Manco-Johnson M. Barriers to compliance with
prophylaxis therapy in haemophilia. Haemophilia : the official journal of the
World Federation of Hemophilia 2001;7:392-6.

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Contact:

Biogen Idec
Media Contact:
Todd Cooper, +1 781 464 3260
public.affairs@biogenidec.com
or
Investor Relations Contact:
Ben Strain, +1 781 464 2442
IR@biogenidec.com
or
Swedish Orphan Biovitrum
Media Contact:
Oskar Bosson, +46 70410 71 80
Head of Communications
or
Analyst/Investor Contact:
Jörgen Winroth, +46 8 697 2135
Vice President, Head of Investor Relations
 
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