Open Label Two-Arm Study to Evaluate Rilzabrutinib in IgG4-Related Disease Participants

An Open Label, Two-Arm Study to Evaluate the Effect of Rilzabrutinib (PRN1008/SAR444671) on Safety and Disease Activity in ParticipantsWith IgG4-Related Disease

This is a Phase 2a, multi-center, open-label, two-arm study of approximately 25 patients with active IgG4-related disease (IgG4-RD). The two arms include (1) Experimental: rilzabrutinib with glucocorticoids and (2) Active Comparator: glucocorticoids only.

4 weeks of screening, 12 weeks of main treatment, 12 weeks of cross-over (for GC-only group), 40 weeks of extension treatment and 4-week follow-up.

Key Inclusion Criteria:

• Be male or female with age ≥18 years.
• Have a clinical diagnosis of IgG4-RD.
• Be willing to taper off an equivalent prednisone dose of between 20-40 mg/day in 2 weeks.

Key Exclusion Criteria:

• Currently or within 6 months of screening taking rituximab, other B-cell depleting agents, or alkylating agents unless B cell concentrations have been demonstrated by flow cytometry to return to normal values (defined as 5 cells per cubic mm).
• History of solid organ transplant
• Positive at Screening for HIV, hepatitis B, hepatitis C, or TB
• Female patients who are pregnant or nursing.
• NOTE: Other Inclusion/Exclusion criteria may apply.