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American Association of Neuromuscular & Electrodiagnostic Medicine (AANEM) Annual Meeting | 2024 | iPharmaCenter

Updated: Oct 17

Nipocalimab Shows Long-Term Disease Control in Adolescents with Generalized Myasthenia Gravis in Phase 2/3 Trial

The first FcRn inhibitor to exhibit prolonged disease control over a 24-week period in antibody-positive adolescents aged 12-17, expanding the demographic in which nipocalimab has been tested.


Johnson & Johnson reported findings from the Phase 2/3 Vibrance-MG clinical trial, assessing nipocalimab in adolescents (12-17 years old) with generalized myasthenia gravis (gMG) who were anti-AChR antibody positive.


Patients receiving a combination of nipocalimab and standard care (SOC) maintained disease control, as indicated by the primary endpoint of reduced immunoglobulin G (IgG) levels from baseline over a 24-week period. Secondary outcomes included improvements in MG-ADL (Myasthenia Gravis Activities of Daily Living) and QMG (Quantitative Myasthenia Gravis) scores.



Approximately 10% of myasthenia gravis cases are diagnosed in adolescents, and the disease tends to be more severe in younger patients. Nearly 43% of pediatric patients have been hospitalized more than five times, with 46% having required at least one stay in an intensive care unit, and 68% experiencing exacerbations of the disease.


The combination of nipocalimab and SOC successfully achieved the trial’s primary goal of reducing total serum IgG by 69%.

Additionally, it met two key secondary goals, reflecting improvement in MG-ADL and QMG scores. By the end of the treatment phase, four out of five participants reached minimal symptom expression, defined as an MG-ADL score between 0 and 1.



Nipocalimab was well tolerated over the six-month treatment period, showing similar safety results to those observed in adults during the Vivacity-MG study. There were no significant adverse events or treatment discontinuations.


These Phase 2/3 findings in adolescents, revealed for the first time, align with outcomes from earlier research involving adults with gMG. Nipocalimab, when used alongside SOC, is the first FcRn blocker to show prolonged disease control in a pivotal trial, as measured by enhanced MG-ADL scores compared to placebo over a six-month period of regular biweekly dosing in adult patients with gMG.

 

Amgen shares promising Phase 3 results for Uplizna (inebilizumab-cdon) in generalized myasthenia gravis (GMG) at AANEM 2024

The MINT study demonstrates significant and meaningful improvements in both AChR+ and MuSK+ patients.

Amgen recently announced favorable results from its Phase 3 MINT trial, which evaluated the effectiveness and safety of Uplizna (inebilizumab) for treating adults with generalized myasthenia gravis (gMG), a rare autoimmune disorder.


The trial reached its primary objective, showing a statistically significant improvement in Myasthenia Gravis Activities of Daily Living (MG-ADL) score with Uplizna (-4.2) versus placebo (-2.2) at Week 26. This difference was highly significant across the combined patient group, which included those with acetylcholine receptor autoantibody-positive (AChR+) and muscle-specific kinase autoantibody-positive (MuSK+) conditions. Participants were administered Uplizna or a placebo on Day 1 and Day 15. Uplizna continued to show improvement throughout the 26-week period.



Furthermore, patients who were on corticosteroids at the study’s start had their dosage gradually reduced beginning in Week 4, with most tapering down to 5 mg of prednisone daily by Week 24. No new safety concerns were noted.


Secondary goals of the trial were evaluated sequentially, with Uplizna showing significant and clinically important benefits compared to placebo across four key endpoints:

  • Uplizna showed a notable improvement in Quantitative Myasthenia Gravis (QMG) score for the entire population (-4.8) compared to placebo (-2.3) at Week 26 (p=0.0002).

  • Among AChR+ patients, Uplizna resulted in a mean MG-ADL score reduction of -4.2, versus -2.4 in the placebo group (p=0.0015).

  • In AChR+ patients, the QMG score improvement with Uplizna was -4.4 compared to -2.0 with placebo (p=0.0011).

  • For MuSK+ patients, Uplizna produced a reduction in MG-ADL score of -3.9, compared to -1.7 for placebo (p=0.0297).


Although the MuSK+ group showed a trend favoring Uplizna in QMG score changes, the difference (-5.2 for Uplizna versus -3.0 for placebo) was not statistically significant.

Safety outcomes during the placebo-controlled phase were consistent with Uplizna’s established safety profile. The most frequently observed side effects included COVID-19, nasopharyngitis, urinary tract infections, infusion-related reactions, headaches, and cough in the combined group.



MINT is the largest placebo-controlled trial conducted for a biologic treatment in gMG, involving 238 adults, including 48 MuSK+ patients and 190 AChR+ patients. Additional data over a 12-month period will further describe Uplizna’s efficacy and safety in AChR+ gMG patients.


At the 2024 AANEM meeting, Amgen will also showcase research highlighting the burden of glucocorticoid use among gMG patients in the U.S., focusing on the associated side effects and the increased healthcare costs due to higher glucocorticoid use.


Uplizna is already approved in multiple countries for treating neuromyelitis optica spectrum disorder (NMOSD) in adults who are anti-aquaporin-4 (AQP4) antibody positive. The U.S. Food and Drug Administration (FDA) also recently granted Uplizna Breakthrough Therapy Designation for IgG4-related diseases, following promising Phase 3 data released in June.

Amgen plans to seek U.S. approval based on the MINT trial results, with plans to expand to additional key markets shortly thereafter.



About the MINT trial

The MINT trial is a Phase 3, randomized, double-blind, placebo-controlled study (NCT04524273) assessing Uplizna's safety and effectiveness in adults with gMG. The study enrolled 238 participants, including 190 with AChR+ and 48 with MuSK+. Eligibility criteria included MGFA classification II, III, or IV, MG-ADL scores between 6 and 10, or an MG-ADL score of at least 11, and a QMG score of at least 11. Participants could also be using corticosteroids or non-steroidal immunosuppressants. The trial's primary endpoint measured changes in MG-ADL scores at Week 26, while secondary endpoints included changes in QMG scores, MG-ADL scores in AChR+ and MuSK+ populations, and the tapering of corticosteroid doses. An optional three-year open-label treatment phase is also included in the trial.


About generalized myasthenia gravis (gMG)Generalized myasthenia gravis (gMG) is a rare, chronic autoimmune disease in which B-cells attack the neuromuscular junction, leading to muscle weakness, breathing difficulties, trouble swallowing, and issues with speech and vision. Around 85% of those diagnosed with myasthenia gravis have gMG.

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