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      EMEA/Newsroom/Immunology/Johnson & Johnson seeks first EU approval of nipocalimab to treat a broad population of patients living with antibody-positive generalised myasthenia gravis
      Immunology

      Johnson & Johnson seeks first EU approval of nipocalimab to treat a broad population of patients living with antibody-positive generalised myasthenia gravis

      Marks first EMA submission for nipocalimab, an investigational treatment that binds with high affinity and specificity to block FcRn and reduce levels of autoantibodies

      Filing based on the Phase 3 Vivacity-MG3 programme, the first registrational study results in the class demonstrating sustained disease control over 24 weeks in antibody-positive adult patients: anti-AChR+, anti-MuSK+, anti-LRP4+

      BEERSE, BELGIUM (September 12, 2024) – Janssen-Cilag International NV, a Johnson & Johnson company, today announced the submission of the Marketing Authorisation Application (MAA) to the European Medicines Agency (EMA) seeking first approval of nipocalimab for the treatment of people living with generalised myasthenia gravis (gMG).

      The application included data from the Phase 3 Vivacity-MG3 study which showed that outcomes for a broad population of antibody-positive participants who received nipocalimab plus standard of care (SOC) were superior compared to those who received placebo plus SOC.1 The primary endpoint of the study measured improvement in the Myasthenia Gravis - Activities of Daily Living (MG-ADL)a score from baseline over 24 weeks and study participants included anti-AChR+, anti-MuSK+, and anti-LRP4+b antibody-positive adults, which account for approximately 95 percent of the gMG patient population, making Vivacity-MG3 the first registrational study to demonstrate sustained disease control in these subtypes.1,2 Safety and tolerability were consistent with other nipocalimab studies.3,4,5,c

      “We are encouraged by the potential of nipocalimab to provide sustained disease control for people living with generalised myasthenia gravis, a chronic, life-long disease,” said Bill Martin, Ph.D., Global Therapeutic Area Head, Neuroscience, Johnson & Johnson Innovative Medicine. “The filing for approval of nipocalimab represents an important step forward as Johnson & Johnson continues to push the boundaries of research to develop innovative solutions to treat autoantibody-driven diseases, building on decades of expertise in neuroscience and immunology. We look forward to working with the EMA in their review of the data supporting the submission.”

      Nipocalimab is the first FcRn blocker to demonstrate sustained disease control measured by improvement in MG-ADL when added to background SOC compared with placebo plus SOC over a period of six months of consistent dosing (every other week).1,6,d

      Editor’s notes:
      a. MG-ADL (Myasthenia Gravis – Activities of Daily Living) provides a rapid clinical assessment of the patient’s recall of symptoms impacting activities of daily living, with a total score range of 0 to 24; a higher score indicates greater symptom severity.7
      b. Positive patients include anti-acetylcholine receptor positive antibody (AChR+), anti-muscle specific tyrosine kinase positive antibody (MuSK+) and/or anti-low density lipoprotein receptor-related protein 4 positive antibody (LRP4+). 1
      c. The overall incidence of adverse events (AEs), serious adverse events (SAEs) and AEs leading to discontinuation were similar to that in the placebo plus SOC group. Specifically, 81.6 percent of patients (n=80) treated with nipocalimab plus SOC experienced AEs, closely matched by 82.7 percent (n=81) in the placebo plus SOC group. Serious AEs were reported by 9.2 percent of patients (n=9) in the nipocalimab plus SOC group compared to 14.3 percent (n=14) in the placebo plus SOC group.1
      d. Patients who received nipocalimab plus current SOC had a mean change of -4.70 [standard error (SE) 0.329]. Patients on placebo plus current SOC had a mean change of -3.25 (SE 0.335); difference of leastsquares (LS) means -1.45 [0.470]; P=0.002.1

      About Generalised Myasthenia Gravis (gMG)
      Myasthenia gravis (MG) is an autoantibody disease in which autoantibodies target proteins at the neuromuscular junction, disrupt neuromuscular signalling, and impair or prevent muscle contraction.8,9 In MG, the immune system mistakenly attacks proteins at the neuromuscular junction, e.g., anti-acetylcholine receptor [AChR], anti-musclespecific tyrosine kinase [MuSK] or anti-lipoprotein-related protein 4 [LRP4], that can block or disrupt normal functioning, preventing signals from transferring from nerves to muscles.10 The disease impacts between 56,000 and 123,000 people in Europe and an estimated 700,000 people worldwide.8,11 The disease affects both men and women and occurs across all ages, racial and ethnic groups, but most frequently impacts young women and older men.12 Roughly 50 percent of individuals diagnosed with MG are women, and about one in five of those women are of childbearing potential.13,14,15

      Initial disease manifestations are usually ocular but in 85 percent or more the disease generalises (gMG), which is characterised by fluctuating weakness of the skeletal muscles leading to symptoms like limb weakness, drooping eyelids, double vision and difficulties with chewing, swallowing, speech, and breathing. 9,16,17 Although gMG may be managed with current SOC therapies, research is needed to develop new treatments for those who may not respond well enough to or tolerate these options.9

      About the Phase 3 Vivacity-MG3 Study
      The Phase 3 Vivacity-MG3 study was specifically designed to measure sustained efficacy and safety with consistent dosing in this unpredictable chronic condition where unmet need remains high. Antibody positive or negative adult gMG patients with insufficient response (MG-ADL ≥6) to ongoing standard of care (SOC) therapy were identified and 199 patients, 153 of which were antibody positive, enrolled in the 24-week double-blind placebo-controlled trial.18,19 Randomisation was 1:1, nipocalimab plus current SOC (30 mg/kg IV loading dose followed by 15 mg/kg every two weeks) or placebo plus current SOC. 19 Baseline demographics were balanced across arms (77 nipocalimab, 76 placebo). 19 The primary endpoint of the study was mean change in MG-ADLb score from baseline over Weeks 22, 23 and 24 in antibody-positive patients. A key secondary endpoint included change in Quantitative Myasthenia Gravis (QMG) score, which is a 13-item assessment by a clinician that quantifies MG disease severity. Long-term safety and efficacy were further assessed in an ongoing OLE phase.18

      About Nipocalimab
      Nipocalimab is an investigational monoclonal antibody, purposefully designed to bind with high affinity to block FcRn and reduce levels of circulating immunoglobulin G (IgG) antibodies, while preserving immune function without causing broad immunosuppression.20 This includes autoantibodies and alloantibodies that underlie multiple conditions across three key segments in the autoantibody space including Rare Autoantibody diseases, Maternal Foetal diseases mediated by maternal alloantibodies and Prevalent Rheumatology. 18,21,22,23,24,25,26,27,28 Blockade of IgG binding to FcRn in the placenta is also believed to prevent transplacental transfer of maternal alloantibodies to the foetus.29

      The European Medicines Agency (EMA) and U.S. Food and Drug Administration (FDA) have granted several key designations to nipocalimab including:

      • EU EMA Orphan medicinal product designation for HDFN in October 2019
      • U.S. FDA Fast Track designation in haemolytic disease of the foetus and newborn (HDFN) and warm autoimmune haemolytic anaemia (wAIHA) in July 2019, gMG in December 2021 and foetal neonatal alloimmune thrombocytopenia (FNAIT) in March 2024
      • U.S. FDA Orphan drug status for wAIHA in December 2019, HDFN in June 2020, gMG in February 2021, chronic inflammatory demyelinating polyneuropathy (CIDP) in October 2021 and FNAIT in December 2023
      • U.S. FDA Breakthrough Therapy designation for HDFN in February 2024

      About Johnson & Johnson
      At Johnson & Johnson, we believe health is everything. Our strength in healthcare innovation empowers us to build a world where complex diseases are prevented, treated, and cured, where treatments are smarter and less invasive, and solutions are personal. Through our expertise in Innovative Medicine and MedTech, we are uniquely positioned to innovate across the full spectrum of healthcare solutions today to deliver the breakthroughs of tomorrow, and profoundly impact health for humanity.

      Learn more at www.janssen.com/emea.

      Follow us at http://www.linkedin.com/company/jnj-innovative-medicine-emea/.

      Janssen-Cilag International NV and Janssen-Cilag Limited are both Johnson & Johnson companies.

      Cautions Concerning Forward-Looking Statements
      This press release contains “forward-looking statements” as defined in the Private Securities Litigation Reform Act of 1995 regarding product development and the potential benefits and treatment impact of amivantamab and lazertinib. The reader is cautioned not to rely on these forward-looking statements. These statements are based on current expectations of future events. If underlying assumptions prove inaccurate or known or unknown risks or uncertainties materialise, actual results could vary materially from the expectations and projections of Janssen Research & Development, LLC, Janssen Biotech, Inc., Janssen-Cilag, S.A. and JanssenCilag International NV, and/or Johnson & Johnson. Risks and uncertainties include, but are not limited to: challenges and uncertainties inherent in product research and development, including the uncertainty of clinical success and of obtaining regulatory approvals; uncertainty of commercial success; manufacturing difficulties and delays; competition, including technological advances, new products and patents attained by competitors; challenges to patents; product efficacy or safety concerns resulting in product recalls or regulatory action; changes in behaviour and spending patterns of purchasers of health care products and services; changes to applicable laws and regulations, including global health care reforms; and trends toward health care cost containment. A further list and descriptions of these risks, uncertainties and other factors can be found in Johnson & Johnson’s Annual Report on Form 10-K for the fiscal year ended December 31, 2023, including in the sections captioned “Cautionary Note Regarding ForwardLooking Statements” and “Item 1A. Risk Factors,” and in Johnson & Johnson’s subsequent Quarterly Reports on Form 10-Q and other filings with the Securities and Exchange Commission. Copies of these filings are available online at http://www.sec.gov/, http://www.jnj.com/ or on request from Johnson & Johnson. None of Janssen Research & Development, LLC, Janssen Biotech, Inc., Janssen-Cilag, S.A. and Janssen-Cilag International NV, nor Johnson & Johnson undertakes to update any forward-looking statement as a result of new information or future events or developments.

      # # #

      Media contact:
      Alexandra Nisipeanu
      [email protected]
      +40 744-383-413

      Investor contact:
      Raychel Kruper
      [email protected]

      CP-475639
      September 2024

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      2 J&J Data on file (RF-421587). Approximate incidence of seronegativity in generalised myasthenia gravis (gMG). Date of preparation: June 2024.
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