Cinryze data on hereditary angioedema presented at EAACI 2011

Jun 14 2011

ViroPharma Incorporated (NASDAQ: VPHM) today announced that data relating to Cinryze® (C1 esterase inhibitor [human]) from nine accepted abstracts were presented as poster presentations at the 30th Congress of the European Academy of Allergy and Clinical Immunology (EAACI), June 11 through 15, in Istanbul, Turkey.  Of the nine posters, two contain new data and seven contain data previously presented at the 2010 International Scientific Conference of the World Allergy Organization (WAO), the 2010 Annual Meeting of the American College of Allergy, Asthma & Immunology (ACAAI), and the 2011 American Academy of Allergy Asthma & Immunology (AAAAI) Annual Meeting.

Cinryze is the first and only U.S. FDA-approved C1 esterase inhibitor therapy indicated for routine prophylaxis against angioedema attacks in adolescent and adult patients with hereditary angioedema (HAE), a rare, debilitating and potentially fatal disease.  Currently, Cinryze is available only in the United States, and is not approved to treat acute angioedema attacks, for children with HAE, or for pre-procedural administration.

In Europe, the Committee for Medicinal Products for Human Use (CHMP) has adopted a positive opinion for Cinryze for treatment and pre-procedural prevention of angioedema attacks in adults and adolescents with hereditary angioedema (HAE), and routine prevention of angioedema attacks in adults and adolescents with severe and recurrent attacks of hereditary angioedema (HAE), who are intolerant to or insufficiently protected by oral prevention treatments or patients who are inadequately managed with repeated acute treatment.  

HAE is a rare, debilitating and potentially life-threatening genetic disorder that affects at least 10,000 people in Europe.  HAE is a variable disease due to a deficiency of C1 inhibitor, a human plasma protein that prevents swelling. Patients can experience unpredictable, recurrent, disabling and potentially deadly attacks of swelling that can affect the larynx, abdomen, face, extremities and genitourinary tract.  If approved, Cinryze would be the first C1 esterase inhibitor product approved for preventative therapy of HAE across all of Europe.  

"As we move closer toward providing Cinryze for HAE patients in Europe, it is important that we expand the awareness among the medical community of the available data on clinical experience with this important drug for patients with hereditary angioedema," commented Mark Sampson, MD, ViroPharma's vice president of clinical development and medical affairs, Europe.  "HAE is a variable disease that can be seen by various physician specialties, and Cinryze is poised to be a comprehensive management option for European physicians and patients."

EAACI Poster Presentations (new data)

In a poster entitled, 'Use of Nanofiltered C1 Esterase Inhibitor (Human) [C1 INH-nf] for the Treatment of Gastrointestinal (GI) Attacks in Subjects with Hereditary Angioedema (HAE),' Dr. William Lumry, M.D., of the University of Texas Southwestern Medical School in Dallas, Texas discussed the potential for Cinryze to treat GI-localized HAE attacks.  These data were compiled from an open-label, multicenter (29 sites) study that enrolled 113 subjects with a diagnosis of HAE.  Gastrointestinal attacks represented the largest proportion (351/598, 59 percent) of HAE attacks in this study.  Subjects received treatment with C1 INH-nf 1000U IV and could receive a second dose of C1 INH-nf 1000U if their symptoms had not improved by 60 minutes.  The results cited on the poster included the following:

• Seventy-seven subjects experienced a total of 351 GI attacks, with 97 percent (339/351) achieving relief within 4 hours after C1 INH-nf administration; median time to beginning of relief was 30 minutes.  This response time was comparable to the overall median response time for all attacks at all anatomic locations in this study (30 minutes).
• Among subjects treated for greater than one GI attack, the efficacy of C1 INH-nf did not diminish with subsequent repeated administration.
• Of the 113 subjects enrolled, the most common (three to five percent of subjects) adverse events were sinusitis, nasopharyngitis, streptococcal pharyngitis, HAE, constipation, cough, rash, and bronchitis.
• There were no hypersensitivity reactions, including anaphylaxis, related to C1 INH-nf. HBV, HCV, and HIV testing revealed no evidence of viral transmission. There was no evidence of development of clinically relevant anti-C1 INH antibodies.

In a poster entitled, 'Use of Nanofiltered C1 Esterase Inhibitor (Human) [C1 INH-nf] for the Treatment of Extremity and Facial Attacks in Subjects with Hereditary Angioedema (HAE),' Dr. Marc Riedl, M.D., of the University of California, Los Angeles, David Geffen School of Medicine presented the experience of 51 subjects who received C1 INH-nf to treat 156 facial and extremity attacks of HAE.  Cutaneous attacks of the extremity>

• Ninety six percent of these attacks (149/156) achieved relief within 4 hours after C1 INH-nf administration; median time to beginning of relief was 30 minutes.  This response time was comparable to the overall median response time for all attacks at all anatomic locations in this study (30 minutes).
• The efficacy of C1 INH-nf did not diminish with subsequent repeated administration. Of the 113 subjects enrolled, the most common (three to five percent of subjects) adverse events were sinusitis, nasopharyngitis, streptococcal pharyngitis, HAE, constipation, cough, rash, and bronchitis.
• There were no hypersensitivity reactions, including anaphylaxis, related to nf-C1 INH. HBV, HCV, and HIV testing revealed no evidence of viral transmission. There was no evidence of development of clinically relevant anti-C1 INH antibodies.

Additional EAACI Poster Presentations (previously presented data)

1. Open-Label Use of Nanofiltered C1 Esterase Inhibitor (Human) (C1 INH-nf) for the Treatment of Hereditary Angioedema (HAE) Attacks
2. Open-Label Use of Nanofiltered C1 Esterase Inhibitor (Human) (C1 INH-nf) for the Prophylaxis of Hereditary Angioedema (HAE) Attacks
3. Pre-procedure Administration of C1 Esterase Inhibitor (Human) (C1 INH-nf) for the Prevention of Hereditary Angioedema (HAE) Attacks after Medical, Dental, or Surgical Procedures
4. Site of Care of Nanofiltered C1 Esterase Inhibitor (Human) (C1 INH-nf) in Patients With Hereditary Angioedema (HAE)
5. Safety and Efficacy of Nanofiltered C1 Esterase Inhibitor (Human) (C1 INH-nf) for the Treatment of Laryngeal Attacks in Subjects with Hereditary Angioedema (HAE)
6. Open-Label Use of Nanofiltered C1 Esterase Inhibitor (Human) (C1 INH-nf) for Treatment of Acute Attacks of Hereditary Angioedema (HAE) in Pediatric Subjects
7. Open-Label Use of Nanofiltered C1 Esterase Inhibitor (Human) (C1 INH-nf) for Treatment or Prophylaxis of Acute Attacks of Hereditary Angioedema (HAE) in Pregnant Subjects