CSL Behring Analysis Shows Berinert® Provides Significant Relief from Cutaneous Attacks of Hereditary Angioedema

MARBURG, GERMANY — 23 February 2013

New data from a CSL Behring analysis show that treatment with Berinert®, C1 Esterase Inhibitor (Human), provides significant resolution of cutaneous attacks of hereditary angioedema (HAE). The data were presented at the 2013 annual meeting of the American Academy of Allergy, Asthma & Immunology (AAAAI). HAE is a rare, potentially fatal swelling disorder caused by a deficiency of C1 Esterase Inhibitor. (C1-INH) Cutaneous HAE attacks are characterized as skin edema or swelling, and often disable and disfigure the face and extremities at the time of the attack.

“While the efficacy of C1 Esterase Inhibitor concentrate in resolving acute laryngeal and abdominal HAE attacks has been well established in the scientific community, data on its effectiveness in treating cutaneous edema is limited ” said Konrad Bork, M.D., Professor of Dermatology, University Hospital, Mainz, Germany. “In this first-ever analysis, we found a highly significant difference in total attack resolution time (TRT) between treatment of peripheral cutaneous attacks of HAE with C1-INH therapy and no treatment. C1-INH provided dramatically faster relief for patients experiencing swelling of the extremities, a painful and frightening condition where a rapid resolution is an important therapeutic goal."

Study Design
Data from 36 HAE patients treated with 20 IU/kg of C1-INH in the International Multicenter Prospective Angioedema C1-INH Trials (I.M.P.A.C.T. 2), who experienced 284 edema (peripheral and facial) attacks, were compared with historical control data from 45 patients, with 8,404 untreated attacks of cutaneous edema from a hospital data base of HAE patients. The study endpoint was the difference in TRT between patient populations, which were demographically comparable according to baseline characteristics, including age, sex and HAE type. The endpoint was assessed using a Wilcoxon test and a log-rank test.

The historical control group had a mean TRT of 3.80 days (90% CI [3.36; 4.24]; median: 3.00 days), in comparison to the patients treated with C1-INH who showed a mean TRT of 1.39 days (90% CI [1.24; 1.55]; median: 1.3 days). Study results showed a significant (p<0.001) difference between treated and untreated cutaneous attacks, which was confirmed by Kaplan-Meier graphs.

About Hereditary Angioedema
HAE is a rare genetic disorder caused by a deficiency of C1-INH. It is inherited in an autosomal dominant manner. Symptoms of HAE include episodes of edema – or swelling – in the face, abdomen, larynx or extremities. Patients who have abdominal attacks of HAE can experience episodes of extreme pain, diarrhea, nausea and vomiting caused by swelling of the intestinal wall. HAE attacks that involve the face or throat can result in upper airway obstruction and death by asphyxiation. Diagnosis of HAE requires a blood test to confirm low levels of C1-INH activity.

About Berinert®
Berinert, C1 Esterase Inhibitor (Human), a pasteurized, nanofiltered, plasma-derived intravenous therapy, treats acute facial, abdominal and laryngeal hereditary angioedema (HAE) symptoms by providing C1-INH-deficient adult and adolescent patients with the missing human protein. Without C1-INH, people with HAE may suffer from recurrent episodes of rapid swelling of areas of the skin and underlying tissues including the face, mouth, throat and abdomen. CSL Behring has marketed its pasteurized and nanofiltered C1-INH concentrate, Berinert, in Germany for more than 30 years. The product is also approved and marketed in 23 other European countries, the United States, Australia, Canada, Israel, Argentina, Japan and South Korea.

Berinert is contraindicated in individuals with a history of life-threatening systemic reactions, including anaphylaxis, to C1 esterase inhibitor preparations. The most serious adverse reaction reported in subjects who received Berinert in clinical studies was an increase in the severity of pain associated with HAE. In placebo-controlled clinical trial, dysgeusia was the most common adverse reaction reported in over 4 percent of subjects who received Berinert and more frequently than in the placebo group. Thrombotic events have been reported in patients receiving C1 esterase inhibitor product, including Berinert, at the recommended dose as well as when used off-label or at higher-than-labeled doses. Berinert is derived from human plasma. The risk of transmission of infectious agents, including viruses and, theoretically, the Creutzfeldt-Jakob disease (CJD) agent, cannot be completely eliminated. Please see full prescribing information for Berinert.

About CSL Behring
CSL Behring is a 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 diseases in the newborn. CSL Behring is a subsidiary of CSL Limited, a biopharmaceutical company with headquarters in Melbourne, Australia. For more information, visit CSL Behring operates one of the world’s largest plasma collection networks, CSL Plasma.


Media Contacts:
Sheila A. Burke
Director, Worldwide Commercial Operations Communications & Public Relations
CSL Behring

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