Autoinflammatory & rare systemic diseases
A phase 2 trial of rilzabrutinib in IgG4-related disease
Authors' conclusion
Rilzabrutinib had an acceptable safety profile in patients with IgG4-related disease who had failed/ intolerant to rituximab and who were rituximab-naïve. Most participants discontinued GCs successfully without disease flares and exhibited sustained disease activity reduction.
Study context
- Population
- Participants with IgG4-RD receiving rilzabrutinib plus a 2-to-4-week glucocorticoid (GC) taper, then rilzabrutinib alone for up to 52 weeks
- Participants
- 27 participants
- Comparison
- Cohort A vs Cohort B
- Cohort An = 13
- Cohort Bn = 14
- Outcome
- Proportion of participants without disease flare from first rilzabrutinib dose until end of treatment; and safety
- Follow-up
- End of treatment
Methodological details52-week, 2 cohorts, single-arm
- 52-week
- 2 cohorts
- Single-arm
Main finding
As reported in the abstract
Flare-free and off GCs/immunosuppressants
Participants
70.4%
19 events
Further findings
| Outcome | Values |
|---|---|
| Decrease in disease activity (IgG4-related disease Responder Index), both cohorts · week 12 | Participants mean 8.3 (SD 5.8) |
| Decrease in disease activity (IgG4-related disease Responder Index), participants completing 52 weeks of rilzabrutinib | Participants mean 10.7 (SD 6.3) |
- Decrease in disease activity (IgG4-related disease Responder Index), participants completing 52 weeks of rilzabrutinib: 14/17 completed 52 weeks
Safety
| Outcome | Values |
|---|---|
| Serious TEAEs | Participants 2 events |
- Serious treatment-emergent adverse events (TEAEs): 1 resulting in death; both judged unrelated to study drug
Most frequent TEAEs
- Diarrhoea
- 40.7% (11/27)
- COVID-19
- 18.5% (5/27)
- Dizziness
- 18.5% (5/27)
Original publication
A phase 2 trial of rilzabrutinib in IgG4-related disease. Ann Rheum Dis. Published 21 July 2026. doi:10.
Composed by AI from the published abstract and checked against it. How Briefs are made →