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CSL Behring Presents New Alpha 1 Antitrypsin Deficiency Treatment Data at Global ERS Congress

Symposium “Transitioning Clinical Data into Patient Care – Recent Real-Life Experiences with Alpha 1”

RAPID and RAPID extension trial data to be featured in poster and oral presentations

MARBURG, Germany — 07 September 2017

Global biotherapeutics leader CSL Behring will host a symposium about Alpha 1 Antitrypsin Deficiency (AATD) on Monday, 11 September, during the 27th International Congress of the European Respiratory Society (ERS) in Milan. Jan Stolk, M.D., Ph.D. and Berend Stoel Ph.D., will chair the symposium that is designed to discuss recent key findings from the RAPID trial programme and how these relate to clinical practice.

“Despite compelling evidence for the efficacy of AAT therapy for AATD, a number of challenges remain,” said Dr. Jan Stolk. “This symposium will facilitate discussions about how this evidence can be enhanced and supported through continued research efforts and how the essential work of AATD registries can contribute to this research.”

In addition to discussions of “real-life experiences” and the importance of registry data, Monday’s symposium will also focus on the latest findings from the RAPID and the RAPID extension trial. “Data from the RAPID programme demonstrated that early intervention with AAT is favourable. A further post hoc analysis now shows that treatment is beneficial in patients with both early and late stage disease.” said Professor Joachim Ficker.

CSL Behring will support the presentation of one oral presentation and three posters throughout the conference.

Finally, as part of the “Walk for Alpha 1 - Every Step Counts” programme, visitors to the CSL Behring exhibition booth (F-06) will have the opportunity to contribute their steps walked throughout the conference. CSL Behring will convert the steps into a donation to Team Alpha-1 Athlete, a sports team working to raise awareness about lung disease and AATD. Alpha-1 Athlete’s, Karen Skålvoll, will be at the CSL Behring booth sharing her story and inspiring attendees to make their steps count.

“CSL Behring is proud to be sharing exciting new clinical data at this year’s ERS congress,” said Lutz Bonacker, Senior Vice President, Europe, CSL Behring. “It’s a representation of our promise to patients living with AATD, to further understand treatment options and support awareness initiatives like Team Alpha-1 Athlete.”

About the RAPID RCT and the RAPID OLE
The RAPID RCT (randomised, controlled trial) 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 RAPID OLE (open label extension), 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 panniculitis, 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 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 CSL Behring
CSL Behring is a global biotherapeutics leader driven by its promise to save lives. Focused on serving patients' needs by using the latest technologies, we develop and deliver innovative therapies that are used to treat coagulation disorders, primary immune deficiencies, hereditary angioedema, inherited respiratory disease, and neurological disorders. The company's products are also used in cardiac surgery, organ transplantation, burn treatment and to prevent haemolytic disease of the newborn.

CSL Behring operates one of the world's largest plasma collection networks, CSL Plasma. The parent company, CSL Limited (ASX:CSL), headquartered in Melbourne, Australia, employs nearly 20,000 people, delivering its life-saving therapies to people in more than 60 countries. For more information visit www.cslbehring.com and follow us on www.Twitter.com/CSLBehring.

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Media Contact
Jennifer Purdue
Office: +1 610 878 4802
Mobile: +1 610 306 9355
Email: jennifer.purdue@cslbehring.com

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