Design of a Phase 2, Multicenter, Randomized, Double-Blind, Placebo-Controlled, Parallel-Group Study of Nipocalimab in Participants with Active Idiopathic Inflammatory Myopathies (SPIREA)
Catherine Najem1, Jagriti Craig1, Lisa Christopher-Stine2, Federico Zazzetti3, Wim Noel4, Sindhu Ramchandren5, Christopher Blango Sr.1, Chetan S. Karyekar1, Rohit Aggarwal6
1Janssen Pharmaceutical company of Johnson & Johnson, 2Medicine and Neurology, Johns Hopkins School of Medicine, 3Janssen Medical Affairs Global Services, LLC, 4Janssen Pharmaceutical, 5Janssen Research & Development, LLC, 6Department of Medicine, University of Pittsburgh
Objective:
SPIREA (NCT05379634) aims to evaluate the efficacy and safety of nipocalimab in patients with Idiopathic inflammatory myopathies (IIM).
Background:
IIM are a rare group of systemic autoimmune diseases characterized by progressive muscular weakness and internal organ involvement, often leading to physical disability and decreased quality of life. Nipocalimab is designed to address the underlying disease pathology by selectively blocking the neonatal Fc receptor to reduce pathogenic autoantibodies. In a phase 2 study of generalized myasthenia gravis (NCT03772587), nipocalimab lowered pathogenic IgG autoantibody levels with significant clinical benefit, acceptable safety, and a favorable benefit-risk profile.
Design/Methods:
SPIREA is a phase 2, double-blind, placebo-controlled, randomized clinical trial enrolling adults (N≈200) with active IIM. The study comprises screening, double-blind treatment, long-term extension, and follow-up periods. Randomized participants are treated every 2 weeks with intravenous nipocalimab or placebo through Week 50. Background oral glucocorticoid (GC) doses will be tapered from Weeks 24–44.
Results:
The primary endpoint is the proportion of participants who achieve at least minimal improvement (≥20) in American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) Total Improvement Score (TIS) at Week 52 and on ≤5 mg/day of oral GC from Weeks 44–52. Secondary endpoints include the proportion of participants who achieve ≥20-point improvement in TIS at Weeks 24 and 52.
Conclusions:
The ongoing SPIREA study evaluating nipocalimab’s safety and efficacy in patients with IIM will help to validate the ACR/EULAR-TIS endpoint in IIM and the role of nipocalimab as a steroid sparing agent in IIM.