Autoinflammatory & rare systemic diseases
A protocol for targeted B-lymphocyte depletion for the treatment of IgG4-related disease
Authors' conclusion
Rituximab administration targeting B-cell depletion for a two-year period is an effective treatment strategy for IgG4-RD and can limit the cumulative glucocorticoid exposure. Flares are uncommon and typically occur in the setting of B-cell repopulation, with good clinical responses to further rituximab administration.
Study context
- Population
- Patients with IgG4-RD diagnosed at Imperial College Healthcare NHS Trust, February 2017–October 2022, with >9 months of follow-up after the first rituximab dose43 analysed
- Participants
- 45 patients
- Outcome
- Treatment response according to the IgG4-RD RI
Methodological detailssingle-centre
- Single-centre
Main finding
As reported in the abstract
Response to rituximab (IgG4-RD RI)
- All patients responded to rituximab therapy according to the IgG4-RD responder index (RI)
Observational data: associations, not effects.
Further findings
Low-dose GC at rituximab induction
- Patients also treated with low-dose GC
- 25/43, 58%
- Median prednisolone dose
- 5 mg daily
Remaining on prednisolone at two years
- Patients remaining on prednisolone (of those on GC at induction)
- 4/25 (16%)
- Median prednisolone dose
- 5 mg daily
Disease flares
- Patients with disease flares
- 11/43 (26%)
- Flares with B-cell repopulation
- 9/11
- Flares without B-cell repopulation (>10 cells/uL)
- 2/11 (18.1%)
Response of flares re-treated with rituximab
- Re-treated flares responding positively
- 7/7, 100%
Original publication
A protocol for targeted B-lymphocyte depletion for the treatment of IgG4-related disease. Rheumatology (Oxford). Published 11 December 2024. doi:10.
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