Hizentra (human immune globulin subcutaneous 20%) / CSL Behring - LARVOL DELTA

Subcutaneous Immunoglobulin (IgPro20) Dose Adjustments for Chronic Inflammatory Demyelinating Polyneuropathy Maintenance Therapy in Clinical Practice (P10-11.024). (PubMed, Neurology) - "To assess dosing of IgPro20 (immune globulin subcutaneous [human], 20% liquid, Hizentra®) in clinical practice in patients with chronic inflammatory demyelinating polyneuropathy (CIDP)...Pulley has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Cabaletta...Dr. Jiang has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Janssen ."

Journal • Retrospective data • Pain

Usage of Spirometry in Managing IgG Therapy in CVID with Airway Disease (clinicaltrials.gov) - P4 | N=22 | Recruiting | Sponsor: University of Alabama at Birmingham | Trial completion date: Dec 2026 ➔ Dec 2027 | Trial primary completion date: Dec 2025 ➔ Dec 2026

Trial completion date • Trial primary completion date • Primary Immunodeficiency • Respiratory Diseases

Subcutaneous Immunoglobulin (IgPro20) Dose Adjustments for Chronic Inflammatory Demyelinating Polyneuropathy Maintenance Therapy in Clinical Practice (AAN 2025) - "Objective:To assess dosing of IgPro20 (immune globulin subcutaneous [human], 20% liquid, Hizentra®) in clinical practice in patients with chronic inflammatory demyelinating polyneuropathy (CIDP).Background:Subcutaneous immunoglobulin (SCIg) approved for maintenance therapy for CIDP, enables consistent Ig levels and improved quality of life compared with intravenous immunoglobulin (IVIg)...These cases demonstrate the flexibility of SCIg treatment in patients with CIDP, highlighting the importance of close follow-up to individualize SCIg therapy and achieve optimal clinical outcomes."

Clinical • Pain

RECLAIIM: A Study to Evaluate the Efficacy, Safety, and Pharmacokinetics of IgPro20 in Adults With Dermatomyositis (DM) (clinicaltrials.gov) - P3 | N=126 | Terminated | Sponsor: CSL Behring | Trial completion date: May 2028 ➔ Dec 2024 | Active, not recruiting ➔ Terminated | Trial primary completion date: May 2028 ➔ Dec 2024; Primary endpoint not attained

Trial completion date • Trial primary completion date • Trial termination • Dermatomyositis • Immunology • Myositis

IgPro20 Infusion Simulation using Prefilled Syringes (AAAAI-WAO 2025) - "This human factor validation study assessed the usability of IgPro20 in Hizentra 50ml pre-filled syringe (PFS) using an infusion pump...Conclusions This study demonstrates that trained PID patients can safely and effectively administer IgPro20 from 50ml PFS. Errors were not generally attributed to design or usability of the PFS, suggesting that these issues could be addressed by providing additional training sessions."

Immunology • Primary Immunodeficiency

A Phase 2 randomised trial of safety and pharmacokinetics of IgPro20 and IgPro10 in patients with diffuse cutaneous systemic sclerosis. (PubMed, Rheumatology (Oxford)) - P2 | "This study shows that in patients with dcSSc, safety, pharmacokinetic and bioavailability profiles of IgPro20, and safety and pharmacokinetics of IgPro10, are similar to those observed in other approved indications."

Journal • P2 data • PK/PD data • Immunology • Scleroderma • Systemic Sclerosis

IVIG Vs SCIG in CIDP (clinicaltrials.gov) - P1 | N=20 | Active, not recruiting | Sponsor: Rutgers, The State University of New Jersey | Recruiting ➔ Active, not recruiting | Trial completion date: Oct 2024 ➔ Dec 2025 | Trial primary completion date: Oct 2024 ➔ Dec 2025

Enrollment closed • Trial completion date • Trial primary completion date • Pain

Indirect Treatment Comparison of Efgartigimod vs Immunoglobulins in Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) (ISPOR-EU 2024) - "Among the studies that met the predefined criteria, an ITC was considered feasible against Privigen, Hizentra, and HyQvia. Despite the limitations of the analysis and the limited comparability of the studies, the ITC results indicate that efgartigimod has a comparable or better efficacy profile than EMA-approved immunoglobulins. Further prospective comparative studies are needed to confirm these results."

Pain

AN UNUSUAL REACTION TO HIZENTRA (SUBCUTANEOUS IMMUNE GLOBULIN (HUMAN) 20% LIQUID) (ACAAI 2024) - "Case Description: A 71-year-old female with Myasthenia Gravis on weekly 60 mg infusions of Immune Globulin Subcutaneous (Human) 20% Liquid started 2 years prior, Parkinson's Disease on Carbidopa/ Levodopa, and history of remote stress-induced urticaria, presented with 6 months of recurring, pruritic, erythematous urticarial rash on her arms and torso appearing within hours of infusions. Hizentra is a 20% solution that contains L-proline and polysorbate 80 whereas Privigen is a 10% solution with L-proline, and both have very low IgA content, <50 mcg/mL and <25 mcg/mL respectively. Urticarial Reaction to Subcutaneous Immune Globulin These recurring, pruritic, erythematous urticarial lesions were present on the arms and torso within hours of subcutaneous immune globulin infusion."

CNS Disorders • Dermatology • Immunology • Infectious Disease • Movement Disorders • Myasthenia Gravis • Novel Coronavirus Disease • Parkinson's Disease • Respiratory Diseases • Urticaria

Outcomes of a patient support programme for subcutaneous immunoglobulin therapy in patients with primary or secondary immunodeficiencies or chronic inflammatory demyelinating polyneuropathy. (PubMed, Intern Med J) - "This is the largest real-world evidence study that describes the effectiveness of SCIg PSPs across three therapeutic disease states. These PSPs can optimise hospital resources such as infusion nurse time and allocation of infusion chairs that were once used for intravenous immunoglobulin infusions, improve patient access to SCIg therapy and enable patients self-administer SCIg outside a hospital environment."

Journal • Pain

Double-blind, Randomized, Placebo-controlled Study Evaluating Efficacy and Safety of IgPro20 in Post-COVID-19 POTS (clinicaltrials.gov) - P3 | N=177 | Recruiting | Sponsor: CSL Behring | Not yet recruiting ➔ Recruiting | Trial primary completion date: Feb 2027 ➔ Sep 2027

Enrollment open • Trial primary completion date • Cardiovascular • Infectious Disease • Novel Coronavirus Disease

Double-blind, Randomized, Placebo-controlled Study Evaluating Efficacy and Safety of IgPro20 in Post-COVID-19 POTS (clinicaltrials.gov) - P3 | N=177 | Not yet recruiting | Sponsor: CSL Behring

New P3 trial • Cardiovascular • Infectious Disease • Novel Coronavirus Disease

RECLAIIM: A Study to Evaluate the Efficacy, Safety, and Pharmacokinetics of IgPro20 in Adults With Dermatomyositis (DM) (clinicaltrials.gov) - P3 | N=126 | Active, not recruiting | Sponsor: CSL Behring | Trial completion date: Nov 2027 ➔ May 2028 | Trial primary completion date: Jun 2024 ➔ May 2028

Trial completion date • Trial primary completion date • Dermatomyositis • Immunology • Myositis

Hizentra® in Inflammatory Neuropathies - pHeNIx Study (clinicaltrials.gov) - P=N/A | N=100 | Recruiting | Sponsor: CSL Behring | Trial completion date: Sep 2024 ➔ Dec 2027 | Trial primary completion date: Sep 2024 ➔ Dec 2027

Trial completion date • Trial primary completion date • Pain

Infusion-related Costs for HyQvia Compared to Hizentra in Chronic Inflammatory Demyelinating Polyradiculoneuropathy (AAN 2024) - "Treatment with fSCIG-10% resulted in total annual savings of $1676 per person with CIDP. Savings are attributed to less-frequent administration, resulting in fewer per-person infusion costs and nursing hours."

Immunology • Pain

Usage of Spirometry in Managing IgG Therapy in CVID With Airway Disease (clinicaltrials.gov) - P4 | N=22 | Recruiting | Sponsor: University of Alabama at Birmingham | Not yet recruiting ➔ Recruiting

Enrollment open • Primary Immunodeficiency • Respiratory Diseases

CSL Behring Announces Availability of Hizentra (Immune Globulin Subcutaneous [Human] 20% Liquid) 10g Prefilled Syringe (PRNewswire) - "CSL Behring...announced the availability of a 10g prefilled syringe for Hizentra^® (Immune Globulin Subcutaneous [Human] 20% Liquid). The Hizentra prefilled syringes provide people living with Primary Immunodeficiency (PI) and Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) the ability to elevate their treatment experience by eliminating the need to draw medication from vials. CSL Behring now has a wide range of prefilled syringe sizes to help meet the individual needs of people living with PI or CIDP including a 1g, 2g, 5g and 10g."

Commercial • Immunology • Inflammation

RECLAIIM: A Study to Evaluate the Efficacy, Safety, and Pharmacokinetics of IgPro20 in Adults With Dermatomyositis (DM) (clinicaltrials.gov) - P3 | N=126 | Recruiting | Sponsor: CSL Behring

Trial completion date • Trial primary completion date • Dermatomyositis • Immunology • Myositis

Passive transfer of hepatitis B core antibody after intravenous immunoglobulin administration in adult patients: a retrospective review (EASL-ILC 2023) - "Because of uncertainly and poor knowledge around baseline testing, all received antiviral treatment consisting of either tenofovir (10%), entecavir (13%) or lamivudine (77%), prior to being initiated on immunosuppressive therapy...The most common HNIg used was Privigen/Hizentra with 30 patients (38%) for both seronegative and seropositive patients. Many patients changed to subcutaneous immunoglobulin (SCIg) administration of HNIg during the Covid-19 pandemic, which included Hizentra and Cuvitru... Passive transfer of hepatitis B core antibody is common after HNIg infusions. Hepatitis B core antibody screening should be considered standard practice for each human normal immunoglobulin administration, effectively improving therapeutic decisions by preventing wrong diagnosis of hepatitis B exposure and therefore preventing unnecessary anti-viral use."

Retrospective data • Review • Hepatitis B • Hepatology • Infectious Disease • Inflammation • Novel Coronavirus Disease

Safety and Efficacy of Hizentra® Following Pediatric Hematopoietic Cell Transplant for Treatment of Primary Immunodeficiencies. (PubMed, J Clin Immunol) - "Hizentra® was discontinued in 15 (41%) patients, most commonly due to recovery of B cell function (n = 11). These data demonstrate that Hizentra® is a safe and effective option in children who have received HCT for PIDD."

Journal • Immunology • Infectious Disease • Pediatrics • Primary Immunodeficiency • Septic Shock • Transplantation

A PRESENTATION OF PEDIATRIC EVANS SYNDROME SECONDARY TO CTLA-4 INSUFFICIENCY (ASPHO 2023) - "She was treated with oral prednisolone and sirolimus...However, subsequent visits found dropping IgG and the patient was started on Hizentra infusions... ITP can sometimes present atypically, such as in Evans syndrome. Development of ITP and AIHA most commonly occurs sequentially, rather than simultaneously, making Evans syndrome a challenging condition to diagnose. Although a rare condition, it is important to recognize in order to manage life-threatening relapses."

Clinical • IO biomarker • Anemia • Autoimmune Hemolytic Anemia • Hematological Disorders • Hematological Malignancies • Immunology • Infectious Disease • Leukemia • Myelodysplastic Syndrome • Oncology • Pediatrics • Respiratory Diseases • Thrombocytopenia • Thrombocytopenic Purpura • CTLA4

CSL Behring Receives FDA Approval for Hizentra (Immune Globulin Subcutaneous [Human] 20% Liquid) 50mL Prefilled Syringe (PRNewswire) - "Global biotechnology leader CSL Behring today announced the U.S. Food and Drug Administration (FDA) approved a 50mL/10gm prefilled syringe for Hizentra® (Immune Globulin Subcutaneous [Human] 20% Liquid). CSL Behring now offers a full range of prefilled syringe sizes to meet the individual needs of people living with Primary Immunodeficiency (PI) or Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)...Hizentra is the most prescribed self-infused subcutaneous immune globulin (SCIg) treatment for PI in the U.S. and the first and only SCIg treatment approved for CIDP. The 50mL prefilled syringe will be available in early 2024, which will allow CSL Behring to manufacture supply to meet anticipated demand."

Launch US • sBLA • Immunology • Inflammation

An Unusually Delayed Presentation of X-Linked Agammaglobulinemia (XLA) (AAAAI 2023) - "He now receives weekly subcutaneous immunoglobulin (Hizentra). Ultimately, BTK protein expression gene testing confirmed the diagnosis of X-linked agammaglobulinemia. Patient has had no further admissions or serious infections."

Allergy • Fatigue • Immunology • Infectious Disease • Pneumococcal Infections • Pneumonia • Primary Immunodeficiency • Pulmonary Disease • Respiratory Diseases

A Pilot Trial To Assess The Feasibility And Efficacy Of SCIG In Patients With MG Exacerbation (SCIG-MG) (clinicaltrials.gov) - P3 | N=26 | Completed | Sponsor: University of Alberta | Recruiting ➔ Completed | Trial completion date: Apr 2024 ➔ Oct 2022 | Trial primary completion date: Apr 2024 ➔ Oct 2022

Trial completion • Trial completion date • Trial primary completion date • CNS Disorders • Myasthenia Gravis

A Budget Impact Model of Maintenance Treatment of Chronic Inflammatory Demyelinating Polyneuropathy with IgPro20 (Hizentra) Relative to Intravenous Immunoglobulin in the United States. (PubMed, Pharmacoecon Open) - "This analysis projects that Hizentra is likely associated with favorable economic benefit compared with IVIG in managing CIDP."

HEOR • Journal • Immune Modulation • Immunology • Inflammation • Pain