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OpenTrials
Completed

NCT Number: NCT04520451

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

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.

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Key information

Conditions

Immunoglobulin G4 Related Disease Autoimmune Diseases Immune System Diseases Immunoglobulin G4-Related Disease

Age range

18 year and older

Sex eligibility

All sexes

Study type

Interventional

Phase

Phase 2

Primary location

Investigational Site Number 12403, Vancouver, Canada

Loading trial locations.

About this study

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.

Who can participate

Healthy volunteers accepted: No

Only the study team can determine whether someone qualifies for participation.

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.

Treatment and study plan

Rilzabrutinib

Drug

oral tablet

Other names: PRN1008/SAR444671

Glucocorticoids

Drug

oral tablet or capsule

Primary outcomes

  1. Proportion of participants who are without disease flare following the first dose of rilzabrutinib until the end of treatment

    Time frame: Up to 64 weeks

    Disease flare is defined as an increase in IgG4-RD responder index (RI) >2 or initiation of rescue treatment.

  2. Incidence of SAE, AE leading to discontinuation and possible glucocorticoid-related AE

    Time frame: Up to 68 weeks

  3. Number of participants with Potentially clinically significant abnormalities (PCSAs) for clinical laboratory tests, vital signs and ECG

    Time frame: Up to 68 weeks

Secondary outcomes

  1. Proportion of participants with reduction from baseline IgG4-RD RI activity score by ≥2 points over time

    Time frame: At Week 52

  2. Proportion of patients with an IgG4-RD RI activity score = 0 at over time

    Time frame: Up to 64 weeks

  3. Level and change from baseline of each subclass of the serological markers over time

    Time frame: Up to 64 weeks

    serum protein electrophoresis to determine IgG subclasses and complement factors C3 and C4

  4. Proportion of participants achieving reduction in baseline serum IgG4 level of 10% over time

    Time frame: Up to 64 weeks

  5. Change from baseline in IgG4-RD RI over time

    Time frame: From baseline up to 64 weeks

  6. Change from baseline in IgG4-RD damage, as recorded on the damage portion of the IgG4-RD RI over time

    Time frame: From baseline up to 64 weeks

Sponsors and collaborators

Lead sponsor

Principia Biopharma, a Sanofi Company

Industry

Collaborators

  • Massachusetts General Hospital

Registry information

Official study title

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

Important dates

Study start
2020
Primary completion
2024
Study completion
2024
First posted
Aug 20, 2020
Registry last updated
Oct 14, 2025

OpenTrials presents study information sourced from ClinicalTrials.gov. The official registry record should be consulted for the latest information.

View the official ClinicalTrials.gov record (opens in a new tab)

This listing is for discovery and informational purposes only. It is not medical advice, does not guarantee that a study is recruiting, and does not determine eligibility. Contact the study team and a qualified healthcare professional when considering participation.

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