Background/Purpose:

To evaluate the therapeutic efficacy and safety of Iguratimod in the treatment of mild IgG4 related disease (IgG4-RD).

Methods: Thirty patients with newly diagnosed mild IgG4-RD were enrolled. One initial dose of diprospan, intramuscular injection, and Iguratimod, 25 mg, twice daily, at 0, 12 and 24 weeks, patients were followed up. Follow up indexes including physician global assessment (PGA), IgG4-RD responder index (IgG4-RD RI), serology and imaging, plasma cytokines and adverse drug effect. Flow cytometry technology was performed for the detection of T, B-cell subsets and plasma cells before and after treatment in patients.

Results: The ages of thirty-four patients with newly diagnosed mild IgG4-RD were 50.83±10.24 years old, and disease duration was 31.5 (8-66) months. The ratio of male to female was 1:1. The IgG4-RD RI of patients assessed at 0 weeks was 10.47±3.98. There was a significant decrease of IgG4-RD RI in patients following up at 12 weeks and 24 weeks, which was 3.60±2.44, 3.13±1.71, P<0.0001. Serum IgG and IgG4 levels at baseline were 22.16±9.54 g/L and 12250(5568-15625) mg/L, respectively. The IgG level at week 12 and week 24 was 15.42±6.15 g/L and 15.43±5.93 g/L respectively, and the IgG4 level was 4725 (2738-8748) mg/L and 6020(2613-11450) mg/L, which showed significantly reduction of serum IgG and IgG4, P values were both <0.0001 respectively. After treatment, CD3+CD8+ T cells, plasmablast/plasma cells (surface markers as CD19+CD24-CD38hi, CD19+CD27hiCD38hi, CD19+IgD-CD38hi), and CD19+IgD-CD38-CD27+ memory B cells all decreased significantly. The percentage of CD19+IgD+CD38+/- naïve B cells increased after treatment. there were no significance of CD4+CXCR5+ ICOS+ and CD4+CXCR5+PD-1+ TFH, CD19+ total B cells, CD19+CD24hiCD38hi regulatory B cells, CD138+CD38+ plasma cells, CD4+IFN-r+ Th1, CD4+IL17-A+ classical Th17 cells, CD4+ IL-17A+IFN-r+ Th1-like Th17 cells before and after treatment with Iguratimod. Of 30 patients, 23(76.7%) patients had complete remission of clinical symptoms, 3(10.0%) patients had partial remission, and 4 (13.3%) patients had no response to Iguratimod treatment. According to adverse drug reaction, 3 had oral ulcers, two had stomach discomfort, 1 had mild hair loss, and others were well tolerated and had no significant adverse drug reaction.

Conclusion: Iguratimod is effective for the treatment of mild IgG4-RD, it can improve the clinical symptoms of patients, reduce the serum IgG and IgG4 levels, and can also reduce the peripheral blood CD3+CD8+ T, especially plasmablasts/plasma cells, memory B cells, and up-regulate naïve B cells. Besides, Iguratimod can also reduce the plasma IFN-γ levels. So we recommend that it can be used as a first-line treatment for some patients with mild IgG4-RD.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/therapeutic-efficacy-and-safety-of-iguratimod-in-treating-mild-igg4-related-diseases/