KING OF PRUSSIA, Pa. – 20 July 2018 – Global biotherapeutics leader CSL Behring today announced that it will support the presentation of 14 scientific posters at the 2018 annual meeting of the Peripheral Nerve Society (PNS). Most notably, neuromuscular medical research from the PATH (Polyneuropathy and Treatment with Hizentra) study, the largest ever Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) trial, will be highlighted and new data will be presented for the first time. In addition, CSL Behring will host a symposium, “Managing CIDP with Immunoglobulin: A Clinician’s Guide."
“The PATH extension study data being presented at PNS highlights our commitment to continuing CIDP research and our promise to deliver innovations that clinicians can use,” said Debra Bensen-Kennedy, MD, Vice President for Medical Affairs, North America, CSL Behring. “For that reason, our symposium focuses on providing health care professionals with real world, practical information on CIDP treatment and response measurement, providing them with tools to deliver new options to their patients.”
Sunday’s Symposium, “Managing CIDP with Immunoglobulin: A Clinician’s guide” (July 22, 6 p.m. EST) will be chaired by Richard A. Lewis (professor of neurology, Cedars Sinai Medical Center, Los Angeles) and will feature a lecture on “Subcutaneous Immunoglobulin in CIDP: The Evidence” by Thomas Harbo (neurologist, Aarhus University Hospital, Aarhus, Denmark), a review of “Practical Aspects in Subcutaneous Immunoglobulin Administration” by Melody Bullock (registered nurse and infusion science specialist, CSL Behring) and a review from Jeffrey A. Allen (neurologist, University of Minnesota, Minneapolis) on “Objectives Measures You Should Use to Monitor CIDP and Treatment Response.”
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]
Hizentra (Immune Globulin Subcutaneous [Human] 20% Liquid), the first 20 percent immunoglobulin developed for subcutaneous use, is registered in over 51 countries and approved to treat certain immune deficiencies. Hizentra, the world's most prescribed subcutaneous immunoglobulin, has a proven track record of safety, efficacy, and tolerability and has over 6 million exposures worldwide since 2010.
Important Safety Information for the U.S.
Hizentra®, Immune Globulin Subcutaneous (Human), 20% Liquid, is indicated for:
- Treatment of primary immunodeficiency (PI) in adults and pediatric patients 2 years and older.
- Maintenance therapy in adults with chronic inflammatory demyelinating polyneuropathy (CIDP) to prevent relapse of neuromuscular disability and impairment.
- Limitation of use: Maintenance therapy in CIDP has been systematically studied for 6 months and for a further 12 months in a follow-up study. Continued maintenance beyond these periods should be individualized based on patient response and need for continued therapy.
For subcutaneous infusion only.
- WARNING: Thrombosis may occur with immune globulin products, including Hizentra. 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.
- For patients at risk of thrombosis, administer Hizentra 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.
Hizentra is contraindicated in patients with a history of anaphylactic or severe systemic reaction to human immune globulin (Ig) or components of Hizentra (eg, polysorbate 80), as well as in patients with immunoglobulin A deficiency with antibodies against IgA and a history of hypersensitivity. Because Hizentra contains L-proline as stabilizer, use in patients with hyperprolinemia is contraindicated.
IgA-deficient patients with anti-IgA antibodies are at greater risk of severe hypersensitivity and anaphylactic reactions. Thrombosis may occur following treatment with Ig products, including Hizentra.
Monitor patients for aseptic meningitis syndrome (AMS), which may occur following treatment with Ig products, including Hizentra. In patients at risk of acute renal failure, monitor renal function, including blood urea nitrogen, serum creatinine and urine output. In addition, monitor patients for clinical signs of hemolysis or pulmonary adverse reactions (eg, transfusion-related acute lung injury [TRALI]).
Hizentra is derived from human blood. 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.
The most common adverse reactions (observed in ≥5% of study subjects) were local infusion-site reactions, as well as headache, diarrhea, fatigue, back pain, nausea, extremity pain, cough, upper respiratory tract infection, rash, pruritus, vomiting, upper abdominal pain, migraine, arthralgia, pain, fall, and nasopharyngitis.
The passive transfer of antibodies can interfere with response to live virus vaccines and lead to misinterpretation of serologic test results.
Full Hizentra prescribing information can be found at http://labeling.cslbehring.com/PI/US/Hizentra/EN/Hizentra-Prescribing-Information.pdf.
Privigen® (Immune Globulin Intravenous [Human] 10% Liquid) is the first and only 10 percent, ready to use, room-temperature stored, liquid intravenous immunoglobulin 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 and in the U.S. since 2017. 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 80 countries around the world for treating these and other rare diseases
Important Safety Information for the U.S.
Privigen®,Immune Globulin Intravenous (Human), 10% Liquid, 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 more than 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.
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[i] Laughlin R.S. et al. Incidence and prevalence of CIDP and the association of diabetes mellitus. Neurology. 7;73(1):39-45.
[ii] American Association of Neuromuscular & Electrodiagnostic Medicine (2017). Chronic Inflammatory Demyelinating Polyneuropathy. http://www.aanem.org/Patients/Disorders/Chronic-Inflammatory-Demyelinating-Polyneuropathy. Accessed March 2018.