Can Maintaining Immunosuppressants Reduce Relapse Rates for Patients with Stable Immunoglobulin G4-Related Disease?

In a multicenter, randomized controlled trial, researchers found that maintaining immunosuppressants (IM), with or without low-dose glucocorticoids (GC), significantly reduced relapse rates in patients with stable immunoglobulin G4-related disease (IgG4-RD) compared with the complete withdrawal of both GC and IM.

IgG4-RD is an immune-mediated, fibroinflammatory condition typically managed with GC for induction treatment. However, the disease's tendency to relapse poses challenges for long-term maintenance treatment. The withdraw IMs and steroid in stable IgG4-RD (WInS IgG4-RD) trial assessed whether discontinuing GC and IM was feasible for patients with stable lgG4-RD.

The trial was an open-label, multicenter, randomized study involving 146 patients with IgG4-RD who had clinically inactive disease for at least 12 months while on GC and IM. Participants were randomized into three groups: complete withdrawal of GC and IM, withdrawal of GC with continued IM, or continued treatment of both GC and IM. The primary outcome measured was the relapse rate within 18 months, and secondary outcomes included changes in the IgG4-RD Responder Index (RI), Physician's Global Assessment (PGA), serum IgG4 and IgG levels, as well as adverse events.

The researchers found that 52.1% of patients who discontinued both GC and IM experienced relapse within 18 months, compared with 14.2% in the GC withdrawal group and 12.2% in the group continuing both treatments (p < .001). Changes in RI and PGA were also significantly higher in the group that discontinued both treatments than in both the GC withdrawal group (p < .001) and the group that continued both treatments (p < .001).

Limitations of the study include its open-label design, which may introduce bias, and the potential for long-term effects beyond the 18-month follow-up period.

“The maintenance of IMs, with or without low-dose GC, was found to be superior to the withdrawal of both therapies in preventing relapse for long-time stable IgG4-RD,” the study authors concluded.

Reference

Peng L, Nie Y, Zhou J, et al. Withdrawal of immunosuppressants and low-dose steroids in patients with stable IgG4-RD (WInS IgG4-RD). Ann Rheum Dis. 2024; 83(5):651-660.doi: 10.1136/ard-2023-224487.