Open Label Two-Arm Study to Evaluate Rilzabrutinib in IgG4-Related Disease Patients
Primary Purpose
Immunoglobulin G4 Related Disease
Status
Active
Phase
Phase 2
Locations
International
Study Type
Interventional
Intervention
rilzabrutinib
Glucocorticoids
Sponsored by
About this trial
This is an interventional treatment trial for Immunoglobulin G4 Related Disease focused on measuring +IgG4, glucocorticoid, IgG4-RD, BTK, Bruton's
Eligibility Criteria
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.
Sites / Locations
- Investigational Site Number 84019
- Investigational Site Number 84016
- Investigational Site Number 84018
- Investigational Site Number 84030
- Investigational Site Number 84036
- Investigational Site Number 12403
- Investigational Site Number 25013
- Investigational Site Number 38016
- Investigational Site Number 72415
Arms of the Study
Arm 1
Arm 2
Arm Type
Experimental
Active Comparator
Arm Label
Rilzabrutinib + glucocorticoids
Glucocorticoids
Arm Description
Rilzabrutinib tablets, 400 mg twice daily from Week 0 to Week 12 plus glucocorticoids (20 to 40 mg/day prednisone equivalent tapered to 0 mg/day within 2 weeks on study)
Glucocorticoids (20 to 40 mg/day prednisone equivalent tapered to 0 mg/day within 12 weeks on study)
Outcomes
Primary Outcome Measures
Proportion of participants who are without disease flare following the first dose of rilzabrutinib.
Disease flare is defined as an increase in IgG4-RD responder index (RI) >2 or initiation of rescue treatment.
Incidence of SAE, AE leading to discontinuation and possible glucocorticoid-related AE
Number of participants with Potentially clinically significant abnormalities (PCSAs) for clinical laboratory tests, vital signs and ECG
Secondary Outcome Measures
Proportion of participants with reduction from baseline IgG4-RD RI activity score ≥2 points at Week 12
Proportion of patients with an IgG4-RD RI activity score = 0 at Week 12
Level and change from baseline of each subclass of the serological markers
serum protein electrophoresis to determine IgG subclasses and complement factors C3 and C4
Proportion of participants achieving reduction in baseline serum IgG4 level of 10% at 12 weeks
Change from baseline in IgG4-RD RI over time
Proportion of participants with no disease flares between Week 4 and Week 12, and between Week 12 and Week 52 (or the end of the treatment extension period) among the participants who have treatment extension
Change from baseline over time in IgG4-RD damage, as recorded on the damage portion of the IgG4-RD RI
Full Information
NCT ID
NCT04520451
First Posted
August 11, 2020
Last Updated
September 6, 2023
Sponsor
Principia Biopharma, a Sanofi Company
Collaborators
Massachusetts General Hospital
1. Study Identification
Unique Protocol Identification Number
NCT04520451
Brief Title
Open Label Two-Arm Study to Evaluate Rilzabrutinib in IgG4-Related Disease Patients
Official Title
An Open Label, Two-Arm Study to Evaluate the Effect of Rilzabrutinib (PRN1008/SAR444671) on Safety and Disease Activity in Patients With IgG4-Related Disease
Study Type
Interventional
2. Study Status
Record Verification Date
September 2023
Overall Recruitment Status
Active, not recruiting
Study Start Date
August 22, 2020 (Actual)
Primary Completion Date
August 30, 2024 (Anticipated)
Study Completion Date
September 27, 2024 (Anticipated)
3. Sponsor/Collaborators
Responsible Party, by Official Title
Sponsor
Name of the Sponsor
Principia Biopharma, a Sanofi Company
Collaborators
Massachusetts General Hospital
4. Oversight
Studies a U.S. FDA-regulated Drug Product
Yes
Studies a U.S. FDA-regulated Device Product
No
Product Manufactured in and Exported from the U.S.
Yes
Data Monitoring Committee
Yes
5. Study Description
Brief Summary
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.
Detailed Description
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.
6. Conditions and Keywords
Primary Disease or Condition Being Studied in the Trial, or the Focus of the Study
Immunoglobulin G4 Related Disease
Keywords
+IgG4, glucocorticoid, IgG4-RD, BTK, Bruton's
7. Study Design
Primary Purpose
Treatment
Study Phase
Phase 2
Interventional Study Model
Parallel Assignment
Masking
None (Open Label)
Allocation
Randomized
Enrollment
27 (Actual)
8. Arms, Groups, and Interventions
Arm Title
Rilzabrutinib + glucocorticoids
Arm Type
Experimental
Arm Description
Rilzabrutinib tablets, 400 mg twice daily from Week 0 to Week 12 plus glucocorticoids (20 to 40 mg/day prednisone equivalent tapered to 0 mg/day within 2 weeks on study)
Arm Title
Glucocorticoids
Arm Type
Active Comparator
Arm Description
Glucocorticoids (20 to 40 mg/day prednisone equivalent tapered to 0 mg/day within 12 weeks on study)
Intervention Type
Drug
Intervention Name(s)
rilzabrutinib
Other Intervention Name(s)
PRN1008/SAR444671
Intervention Description
oral tablet
Intervention Type
Drug
Intervention Name(s)
Glucocorticoids
Intervention Description
oral tablet or capsule
Primary Outcome Measure Information:
Title
Proportion of participants who are without disease flare following the first dose of rilzabrutinib.
Description
Disease flare is defined as an increase in IgG4-RD responder index (RI) >2 or initiation of rescue treatment.
Time Frame
At Week 52
Title
Incidence of SAE, AE leading to discontinuation and possible glucocorticoid-related AE
Time Frame
Up to 68 weeks
Title
Number of participants with Potentially clinically significant abnormalities (PCSAs) for clinical laboratory tests, vital signs and ECG
Time Frame
Up to 68 weeks
Secondary Outcome Measure Information:
Title
Proportion of participants with reduction from baseline IgG4-RD RI activity score ≥2 points at Week 12
Time Frame
At Week 52
Title
Proportion of patients with an IgG4-RD RI activity score = 0 at Week 12
Time Frame
At Week 52
Title
Level and change from baseline of each subclass of the serological markers
Description
serum protein electrophoresis to determine IgG subclasses and complement factors C3 and C4
Time Frame
At Week 52
Title
Proportion of participants achieving reduction in baseline serum IgG4 level of 10% at 12 weeks
Time Frame
At Week 12
Title
Change from baseline in IgG4-RD RI over time
Time Frame
From baseline to Week 52
Title
Proportion of participants with no disease flares between Week 4 and Week 12, and between Week 12 and Week 52 (or the end of the treatment extension period) among the participants who have treatment extension
Time Frame
Until Week 52
Title
Change from baseline over time in IgG4-RD damage, as recorded on the damage portion of the IgG4-RD RI
Time Frame
From baseline to Week 52
10. Eligibility
Sex
All
Minimum Age & Unit of Time
18 Years
Accepts Healthy Volunteers
No
Eligibility Criteria
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.
Facility Information:
Facility Name
Investigational Site Number 84019
City
Stanford
State/Province
California
ZIP/Postal Code
94305
Country
United States
Facility Name
Investigational Site Number 84016
City
Boston
State/Province
Massachusetts
ZIP/Postal Code
02114
Country
United States
Facility Name
Investigational Site Number 84018
City
Detroit
State/Province
Michigan
ZIP/Postal Code
48202
Country
United States
Facility Name
Investigational Site Number 84030
City
New York
State/Province
New York
ZIP/Postal Code
10021
Country
United States
Facility Name
Investigational Site Number 84036
City
Portland
State/Province
Oregon
ZIP/Postal Code
97239
Country
United States
Facility Name
Investigational Site Number 12403
City
Vancouver
ZIP/Postal Code
V6K 2V8
Country
Canada
Facility Name
Investigational Site Number 25013
City
Marseille
Country
France
Facility Name
Investigational Site Number 38016
City
Milan
Country
Italy
Facility Name
Investigational Site Number 72415
City
Barcelona
Country
Spain
12. IPD Sharing Statement
Plan to Share IPD
Yes
IPD Sharing Plan Description
Qualified researchers may request access to patient level data and related study documents including the clinical study report, study protocol with any amendments, blank case report form, statistical analysis plan, and dataset specifications. Patient level data will be anonymized and study documents will be redacted to protect the privacy of trial participants. Further details on Sanofi's data sharing criteria, eligible studies, and process for requesting access can be found at: https://vivli.org
Citations:
PubMed Identifier
30612117
Citation
Wallace ZS, Zhang Y, Perugino CA, Naden R, Choi HK, Stone JH; ACR/EULAR IgG4-RD Classification Criteria Committee. Clinical phenotypes of IgG4-related disease: an analysis of two international cross-sectional cohorts. Ann Rheum Dis. 2019 Mar;78(3):406-412. doi: 10.1136/annrheumdis-2018-214603. Epub 2019 Jan 5.
Results Reference
background
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Open Label Two-Arm Study to Evaluate Rilzabrutinib in IgG4-Related Disease Patients
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