https://orcid.org/0000-0002-8297-556X
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Matthew Colquhoun, Tara D Barwick, Eva Bolton, Norma Gibbons, Archie Hughes-Hallett, Jeremy B Levy, Stephen P McAdoo, Constantinos A Parisinos, Natalie Philips, Frederick W K Tam, Florian Wernig, Taryn Youngstein, James A P Tomlinson, A protocol for targeted B-lymphocyte depletion for the treatment of IgG4-related disease, Rheumatology, Volume 64, Issue 5, May 2025, Pages 2847–2854, https://doi.org/10.1093/rheumatology/keae675
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Abstract
To determine the clinical outcomes of patients with immunoglobulin 4-related disease (IgG4-RD) treated with a defined B-cell depletion protocol using rituximab.
Patients were included if they had (i) an IgG4-RD diagnosis at Imperial College Healthcare NHS Trust between February 2017 and October 2022, and (ii) >9 months of follow-up data available following the first rituximab dose. The rituximab protocol targeted B-cell depletion to <10 cells/microliter for a maintenance period of two years. Electronic records were used to define patient demographics, serological and radiological variables and treatment responses according to the IgG4-RD responder index (RI).
Forty-five patients received induction treatment with rituximab. Two patients had insufficient follow-up data for outcome analysis. All patients responded to rituximab therapy according to the IgG4-RD RI. Most patients (25/43, 58%) were also treated with low-dose glucocorticoids at the time of rituximab induction (median prednisolone dose 5 mg daily) and 4/25 (16%) remained on prednisolone at two years (median prednisolone dose 5 mg daily). Disease flares occurred in 11/43 (26%) patients; 9/11 flares occurred in the presence of B-cell repopulation; 2/11 (18.1%) flares occurred in the absence of B-cell repopulation (>10 cells/uL). All flares re-treated with rituximab (7/7, 100%) responded positively.
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.
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