CSL Behring Announces FDA Approval of Privigen® [Immune Globulin Intravenous (Human), 10% Liquid] for the Treatment of Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) in Adults

KING OF PRUSSIA, Pa., Sept. 14, 2017 /PRNewswire/ -- Global biotherapeutics leader CSL Behring today announced that the U.S. Food and Drug Administration (FDA) has approved Privigen^® [Immune Globulin Intravenous (Human), 10% Liquid] for the treatment of adults with chronic inflammatory demyelinating polyneuropathy (CIDP) to improve neuromuscular disability. CIDP is a rare autoimmune disorder that affects the peripheral nerves and may cause permanent nerve damage.

"The FDA approval of Privigen for CIDP represents a significant milestone for individuals with this debilitating and progressive disease. It is a testament to our commitment to meet the needs of patients with disabling neurologic conditions, including CIDP," said Dr. Andrew Cuthbertson, chief scientific officer and director of Research and Development for CSL Limited. "As we focus on building a leading neurology franchise, we continue to advance clinical research to determine innovative and improved uses of immunoglobulin therapy that can benefit patients and improve their quality of life."

The FDA approval was based on results from two Phase III clinical studies that focused on the use of immunoglobulin (Ig) therapy for treating CIDP – the Polyneuropathy And Treatment with Hizentra (PATH) study, the largest controlled clinical study in CIDP patients to date, and the Privigen Impact on Mobility and Autonomy (PRIMA) study. In PATH, 207 patients receiving Privigen were studied for up to 13 weeks, and 73 percent responded to Privigen over the course of treatment, as measured by their adjusted score on the Inflammatory Neuropathy Cause and Treatment (INCAT) scale, which measures the ability to walk and perform tasks. In PRIMA (n=28), 61 percent of patients responded to Privigen over 25 weeks, as measured by their adjusted INCAT score.

"It is a priority in the care of CIDP patients to provide therapies that improve and maintain strength and function while at the same preventing relapses and minimizing side effects. However, current treatments do not work for all CIDP patients," said Dr. Mazen M. Dimachkie, Professor and Director of Neuromuscular Division, Executive Vice Chairman, Department of Neurology at the University of Kansas Medical Center and an investigator in the PATH study. "Privigen's approval by the FDA for the treatment of CIDP means that people with CIDP and their treating physicians have gained another treatment option that is safe and effective in helping improve strength and motor function, while potentially delaying disease relapse."

"People living with CIDP can experience a progression of their disease, which may result in tingling, muscle weakness, fatigue and other symptoms that limit their daily activities and decrease their quality of life," said Lisa Butler, executive director of the GBS|CIDP Foundation International. "The approval of this intravenous immunoglobulin to improve disability represents an important treatment advance for the patients, caregivers and families who are struggling with CIDP. We are grateful for CSL Behring's efforts in this area and their commitment to advancing patient care for the CIDP community."

For more information about Privigen, including the U.S. Prescribing Information, visit www.privigen.com.

About CIDP
In CIDP, a rare autoimmune disorder that affects the peripheral nerves (those outside the brain and spinal cord), the myelin sheath, the protective covering of the nerves, is damaged. This may result in numbness or tingling, muscle weakness, fatigue and other symptoms. CIDP effects can worsen over time, leading to significant activity limitations and a decreased quality of life. CIDP can occur at any age and is more common in men than in women. Approximately 30 percent of CIDP patients will progress to wheelchair dependence if not treated. In the U.S., it is estimated that the incidence of CIDP is up to two patients per 100,000 people each year,ⁱ with a prevalence of 40,000 people affected.ⁱⁱ

About Privigen^®
Privigen is the first and only 10 percent, ready to use, room-temperature stored, liquid IVIG stabilized with proline. A naturally occurring amino acid, proline has been shown to reduce IgG aggregation and dimer formation. Privigen has been approved to treat CIDP in Europe since 2013. In the U.S., Privigen is also approved for primary humoral immunodeficiency (PI) and chronic immune thrombocytopenic purpura (ITP) in patients age 15 years and older. It is available in over 70 countries around the world for treating these and other rare diseases.

Important Safety Information
Immune Globulin Intravenous (Human), 10% Liquid, Privigen^®, is indicated for the treatment of:

• Primary humoral immunodeficiency (PI)
• Chronic immune thrombocytopenic purpura (ITP) in patients age 15 years and older
• Chronic inflammatory demyelinating polyneuropathy (CIDP) in adults
• • Limitation of use: maintenance therapy in CIDP has not been studied for periods longer than 6 months. Individualize duration of treatment beyond 6 months based on patient response.

WARNING: THROMBOSIS, RENAL DYSFUNCTION AND ACUTE RENAL FAILURE

• Thrombosis may occur with immune globulin products, including Privigen. Risk factors may include advanced age, prolonged immobilization, hypercoagulable conditions, history of venous or arterial thrombosis, use of estrogens, indwelling vascular catheters, hyperviscosity, and cardiovascular risk factors.
• Renal dysfunction, acute renal failure, osmotic nephrosis, and death may occur with immune globulin intravenous (IGIV) products in predisposed patients. Renal dysfunction and acute renal failure occur more commonly in patients receiving IGIV products that contain sucrose. Privigen does not contain sucrose.
• For patients at risk of thrombosis, renal dysfunction or renal failure, administer Privigen at the minimum dose and infusion rate practicable. Ensure adequate hydration in patients before administration. Monitor for signs and symptoms of thrombosis and assess blood viscosity in patients at risk for hyperviscosity.

See full prescribing information for complete boxed warning.

Privigen is contraindicated in patients with history of anaphylactic or severe systemic reaction to human immune globulin, in patients with hyperprolinemia, and in IgA-deficient patients with antibodies to IgA and a history of hypersensitivity.

In patients at risk of developing acute renal failure, monitor urine output and renal function, including blood urea nitrogen and serum creatinine.

Hyperproteinemia, increased serum viscosity, or hyponatremia can occur with Privigen. Infrequently, aseptic meningitis syndrome (AMS) may occur—especially with high doses or rapid infusion.

Hemolysis, either intravascular or due to enhanced red blood cell sequestration, may occur. Risk factors include non-O blood group and high doses. Closely monitor patients for hemolysis and hemolytic anemia.

During and shortly following Privigen infusion, elevations of systolic and diastolic blood pressure (including cases of hypertensive urgency) have been observed. These elevations resolved or significantly improved within hours with oral anti-hypertensive therapy or observation alone. Check patients for a history of hypertension and monitor blood pressure during this period.

Consider relative risks and benefits before prescribing high-dose regimen for chronic ITP and CIDP in patients at increased risk of thrombosis, hemolysis, acute kidney injury or volume overload. Monitor patients for pulmonary adverse reactions (transfusion-related acute lung injury [TRALI]).

Privigen is derived from human plasma. The risk of transmission of infectious agents, including viruses and, theoretically, the Creutzfeldt-Jakob disease (CJD) agent and its variant (vCJD), cannot be completely eliminated.

In clinical studies of patients with PI, the most common adverse reactions to Privigen, observed in >5% of subjects, were headache, fatigue, nausea, chills, vomiting, back pain, pain, elevated body temperature, abdominal pain, diarrhea, cough, stomach discomfort, chest pain, joint swelling/effusion, influenza-like illness, pharyngolaryngeal pain, urticaria, and dizziness. Serious adverse reactions were hypersensitivity, chills, fatigue, dizziness, and increased body temperature.

In clinical studies of patients being treated for chronic ITP, the most common adverse reactions, seen in >5% of subjects, were laboratory findings consistent with hemolysis, headache, elevated body temperature, anemia, nausea, and vomiting. A serious adverse reaction was aseptic meningitis syndrome.

In clinical studies of patients being treated for CIDP, the most common reactions, observed in >5% of subjects, were headache, asthenia, hypertension, nausea, pain in extremity, hemolysis, influenza-like illness, leukopenia, and rash.  Serious adverse reactions were hemolysis, exacerbation of CIDP, acute rash, increased diastolic blood pressure, hypersensitivity, pulmonary embolism, respiratory failure, and migraine.

Treatment with Privigen might interfere with a patient's response to live virus vaccines and could lead to misinterpretation of serologic testing. In patients over 65 and those at risk of renal insufficiency, do not exceed recommended dose and infuse at the minimum rate practicable.

Full Privigen prescribing information, including the complete boxed warning, can be found at http://www.privigen.com/prescribing-information .

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 hemolytic 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, and delivers 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.

ⁱ https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2707109/
ⁱⁱ http://www.aanem.org/Patients/Disorders/Chronic-Inflammatory-Demyelinating-Polyneuropathy.

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