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Phase 2 N=44 Randomized Double-blind Treatment

Safety,Tolerability,Pharmacokinetics and Efficacy of CFZ533 in Moderate to Severe Myasthenia Gravis

Myasthenia Gravis, Generalized

Enrolled (actual)
44
Serious AEs
25.0%
Results posted
Jul 2019
Primary outcome: Primary: Mean Change From Baseline in the Quantitative Myastenia Gravis (QMG) Score at Week 25. Posterior Median Was Used as Measure Type. — -4.07; -2.93 score

Study Design & Population

Study type
Interventional
Phase
Phase 2
Interventions
Placebo (Drug); CFZ533 (Drug)
Age
Adult, Older Adult · 18+ yrs
Sex
All
Sponsor
Novartis Pharmaceuticals
Primary completion
Jul 2017

Outcome Measures

OutcomeResultp-value
PRIMARY
Mean Change From Baseline in the Quantitative Myastenia Gravis (QMG) Score at Week 25. Posterior Median Was Used as Measure Type.
-4.07; -2.93
SECONDARY
Mean Changes From Baseline in the Myasthenia Gravis Composite (MGC) Score. Posterior Median Was Used as Measure Type.
-8.00; -5.62
SECONDARY
Proportion of Patients With Improvement or Worsening by ≥ 3 Points in the QMG Score
10; 9; 2; 2
SECONDARY
Proportion of Patients Intolerant to Steroid Taper
NA; NA
SECONDARY
Number of Patients Who Discontinued Due to Inefficacy or Worsening
0; 0
SECONDARY
Mean Change From Baseline in the Myasthenia Gravis-specific Activities of Daily Living Scale (MG-ADL)
-2.6; -1.1
SECONDARY
Mean Changes From Baseline in the QMG Score at Week 49
-2.9; -2.6
SECONDARY
Mean Change From Baseline in the Myasthenia Gravis Quality of Life (MG QOL-15)
-9.7; -6.7
SECONDARY
Free CD40 on B Cells
34242.9; 31025.9; 5259.1; 24908.3
SECONDARY
Total Soluble CD40 (sCD40) in Plasma
0.1778; 0.1577; 191.1278; 0.1163
SECONDARY
Plasma CFZ533 Concentration at Steady State Conditions (Week 17)
120

Summary

The purpose of this study is to evaluate safety, tolerability, pharmacokinetics/pharmacodynamics and efficacy of CFZ533 as an add-on therapy to standard of care in patients with moderate to severe myasthenia gravis (MG).

Eligibility Criteria

Inclusion Criteria

  • Diagnosis of MG class IIa to IVa inclusive (Myasthenia Gravis Foundation of America Clinical Classification).
  • Quantitative Myasthenia Gravis (QMG) score of 10 or greater. If the QMG score is < 15 no more than 4 points may be derived from items 1 or 2 (ocular motility disturbance and ptosis).
  • Documented history of acetylcholine receptor (AChR) or Muscle Specific Kinase (MuSK) antibody positive.
  • Only one immunosuppressant or immunomodulatory drug at a stable dose is allowed during the study (i) azathioprine and mycophenolate mofetil must be stable for at least 4 months prior to randomization (ii) cyclosporine must be stable for at least 3 months prior to randomization.
  • If the patient is on oral corticosteroids, methotrexate or tacrolimus at screening, the dose must be stable for at least 1 month prior to randomization.
  • If the patient is on cholinesterase inhibitors at screening, the dose must be stable for at least 2 weeks prior to randomization.
  • Women of child-bearing potential, defined as all women physiologically capable of becoming pregnant, may be included in the study if they are using highly effective methods of contraception during the study and for 12 weeks after study treatment.

Exclusion Criteria

  • MGFA grade I, IVb, or V disease.
  • Documented presence of unresected thymoma.
  • Patients having undergone thymectomy or thymo thymectomy (resection of thymoma) within 6 months of screening.
  • Patients having received any of the following treatments prior to randomization:
  • IVIg or plasma exchange within 8 weeks;
  • oral or IV cyclosphosphamide treatment within 3 months;
  • IV corticosteroid bolus (dose higher than 1 mg/kg) within 3 months;
  • belimumab within 6 months. For patients who received belimumab earlier, B cell count should be within normal range;
  • rituximab within 12 months. For patients who received rituximab earlier, B cell count should be within normal range;
  • any other biologic or an investigational drug within 1 month or five times thehalf-life, whichever is longer.
  • Live vaccines within 4 weeks of study drug infusion.
  • Patients who are at significant risk for TE as judged by the investigator or have any one of the following:
  • History of either thrombosis or 3 or more spontaneous abortions with or without the presence of anti-cardiolipin autoantibodies;
  • Presence of prolonged partial thromboplastin time (PTT).
View full record on ClinicalTrials.gov →

Data sourced from ClinicalTrials.gov (NCT02565576). Outcome figures and adverse-event rates are extracted automatically from the registry's posted results and are provided for clinician reference, not as a substitute for the primary publication.

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