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CSL Behring Receives Marketing Authorization for Respreeza® in Europe

Respreeza® is a maintenance treatment for severe Alpha-1 Antitrypsin Deficiency patients and has been shown to slow the progression of emphysema

MARBURG, Germany — 25 August 2015

CSL Behring announced today that the European Commission (EC) has granted marketing authorization in all European Union (EU) member states for Respreeza® to treat patients with Alpha-1 antitrypsin deficiency (AATD). Respreeza®, a highly purified Alpha-1 protein derived from human plasma, is the only Alpha-1 proteinase inhibitor that has been proven in a prospective double blind, placebo controlled trial (the RAPID study) to significantly reduce the loss of lung tissue, slowing the progression of emphysema due to AATD. AATD is a hereditary condition marked by a lack of the Alpha-1 antitrypsin protein, whose main function is to protect the lungs from inflammation.

“AATD is a potentially debilitating disease and many affected individuals suffer from serious lung disease," said Professor Helmut Teschler, MD, Director of the West German Lung Centre at the University of Essen. "With the approval of Respreeza®, healthcare professionals can now provide patients with severe AATD in Europe with a next generation Alpha1-proteinase inhibitor (A1-PI) that provided additional evidence that this augmentation therapy can slow the accelerated loss of lung tissue."

Frank Willersinn, M.D., Alpha-1 Global Steering Committee Chair and patient representative in Europe added, “We are so glad that Respreeza® has been approved by the European Medicines Agency. We commend CSL Behring for their long-lasting commitment to the Alpha-1 community, now bringing their established Alpha 1-antitrypsin product to Europe, allowing it to be a cornerstone for treatment of AATD patients in the near future.”

CSL Behring has marketed A1-PI for 12 years as Zemaira® in the United States, where it is indicated for chronic augmentation and maintenance therapy in adults with A1-PI deficiency and clinical evidence of emphysema. In US, Zemaira® is contraindicated in patients with a history of severe systemic reaction to the product or to A1-PI protein, including anaphylaxis. Due to the risk of severe hypersensitivity, Zemaira® is also contraindicated in immunoglobulin A (IgA)-deficient patients with antibodies against IgA.

About Respreeza®

In EU, Respreeza® is indicated for maintenance treatment, and to slow the progression of emphysema in adults with documented severe A1-PI deficiency (e.g. genotypes PiZZ, PiZ(null), Pi(null,null), PiSZ). Patients are to be under optimal pharmacologic and non-pharmacologic treatment and show evidence of progressive lung disease (e.g. lower forced expiratory volume per second (FEV1) predicted, impaired walking capacity or increased number of exacerbations) as evaluated by a healthcare professional experienced in the treatment of A1-PI deficiency. Respreeza® is contraindicated in patients with hypersensitivity to the active substance or to any of the excipients and IgA deficient patients with known antibodies against IgA, due to the risk of severe hypersensitivity and anaphylactic reactions.

About the RAPID Study

The RAPID study (Randomized, Placebo-controlled Trial of Augmentation Therapy in Alpha-1 Proteinase Inhibitor Deficiency) enrolled 180 severely deficient alpha-1 patients in the U.S. and Europe who were treated for 2 years with Respreeza® or Placebo. The study, published in Lancet in June 2015, showed a significant reduction in the rate of lung tissue loss as measured by CT scan lung density. In those patients who continued into the extension study, where all subjects received Respreeza®, the benefit was maintained for another 2 years.

About Alpha-1 Antitrypsin Deficiency

Alpha-1 Antitrypsin Deficiency is an inherited condition that can result in severe lung disease in adults and liver disease at any age, as well as other less known manifestations such as paniculitis, a skin disease. AATD is the most commonly known genetic risk factor for emphysema and is commonly referred to as genetic COPD. Low levels or absence of the protective protein alpha-1 antitrypsin, which is produced by the liver, characterize AATD.

About CSL Behring

CSL Behring is a global leader in the plasma protein therapeutics industry. Committed to saving lives and improving the quality of life for people with rare and serious diseases, the company manufactures and markets a range of plasma-derived and recombinant therapies worldwide.

CSL Behring therapies are used around the world to treat coagulation disorders including hemophilia and von Willebrand disease, primary immune deficiencies, hereditary angioedema and inherited respiratory disease, and neurological disorders in certain markets. The company’s products are also used in cardiac surgery, organ transplantation, burn treatment and to prevent hemolytic disease of the newborn.

CSL Behring operates one of the world’s largest plasma collection networks, CSL Plasma. CSL Behring is a global biopharmaceutical company and a member of the CSL Group of companies. The parent company, CSL Limited (ASX:CSL), is headquartered in Melbourne, Australia. For more information, visit http://www.cslbehring.com.

Contact:

Jennifer Seiler, External Communications Manager
CSL Behring
O: +1 610-878-4802
jennifer.seiler@cslbehring.com

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