Abstract Details

Title Design of a Phase 2, Multicenter, Randomized, Double-Blind, Placebo-Controlled, Parallel-Group Study of Nipocalimab in Participants with Active Idiopathic Inflammatory Myopathies (SPIREA)

Topic Autoimmune Neurology

Presentation(s) P10 - Poster Session 10 (8:00 AM-9:00 AM)

Poster/Presentation
Number 6-007

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.

Objective SPIREA (NCT05379634) aims to evaluate the efficacy and safety of nipocalimab in patients with Idiopathic inflammatory myopathies (IIM).

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.

Authors/Disclosures
Catherine Najem, PhD PRESENTER	Dr. Najem has received personal compensation for serving as an employee of Janssen R&D US.
	No disclosure on file
Lisa Christopher-Stine, MD, MPH (Johns Hopkins University School of Medicine)	No disclosure on file
	No disclosure on file
Wim Noel (Janssen)	No disclosure on file
Sindhu Ramchandren, MD (Janssen Pharmaceutical Companies of Johnson & Johnson)	Dr. Ramchandren has received personal compensation for serving as an employee of Janssen Pharmaceutical Companies of Johnson & Johnson. Dr. Ramchandren has a non-compensated relationship as a Scientific Advisory Board Member with CMT Research Foundation (CMTRF) that is relevant to AAN interests or activities.
	No disclosure on file
	No disclosure on file
Rohit Aggarwal, MD (Division of Rheumatology, University of Pittsburgh)	Dr. Aggarwal has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Kezar. Dr. Aggarwal has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Pfizer. Dr. Aggarwal has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for AstraZeneca. Dr. Aggarwal has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Alexion. Dr. Aggarwal has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Argenex. Dr. Aggarwal has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for BI. Dr. Aggarwal has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Bristol Myers Squibb. Dr. Aggarwal has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Q32. Dr. Aggarwal has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Corbus. Dr. Aggarwal has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Janssen. The institution of Dr. Aggarwal has received research support from Genentech. The institution of Dr. Aggarwal has received research support from Mallinckodt. The institution of Dr. Aggarwal has received research support from BMS. The institution of Dr. Aggarwal has received research support from EMD Serono. The institution of Dr. Aggarwal has received research support from Q32.