Safety and Efficacy Study of Zilucoplan in Subjects With Immune-Mediated Necrotizing Myopathy

Open to people ages 18-75

• Clinical diagnosis of IMNM (Immune-Mediated Necrotizing Myopathy)
• Positive serology for anti-HMGCR or anti-SRP autoantibodies
• Clinical evidence of weakness (≤ grade 4 out of 5) on manual muscle testing in at least one proximal limb muscle group
• CK (creatine kinase) of >1000 U/L at Screening
• No change in corticosteroid dose for at least 30 days prior to Baseline or anticipated to occur during the first 8-weeks on study
• No changes in immunosuppressive therapy, including dose, for at least 30 days prior to Baseline or anticipated to occur during the first 8-weeks on study

• History of meningococcal disease
• Current or recent systemic infection within 2 weeks prior to Screening or infection requiring intravenous (IV) antibiotics within 4 weeks prior to Screening
• Recent initiation of intravenous immunoglobulin (IVIG) (i.e., first cycle administered less than 90 days prior to Baseline)
• Rituximab use within 90 days prior to Baseline or anticipated to occur during study
• Statin use within 30 days prior to Baseline or anticipated to occur during study
• Plasma exchange within 4 weeks prior to Baseline or expected to occur during the 8-week Treatment Period