Recently, Professor Zhang Wen and Professor Fei Yunyun from the Department of Rheumatology at PUMCH published their study as original research in the top journal “Annals of the Rheumatic Diseases” (a tier 1 journal, or among the top 5%, as ranked by the Chinese Academy of Sciences, with an impact factor of 27.4). The study, an investigator-initiated, multicentre, randomised controlled trial, explored the strategies of withdrawing immunosuppressants and low-dose steroids in patients with stable IgG4-related disease (IgG4-RD). The conclusion is that the maintenance of immunosuppressants (IMs), with or without low-dose glucocorticoid (GC), was found to be superior to the withdrawal of GC+IM in preventing relapse for stable IgG4-RD. The research was supported by the National High Level Hospital Clinical Research Funding.

IgG4-RD is an immune-mediated, inflammatory and fibrotic disease that can affect multiple organs and systems, with complex and varied clinical manifestations. It was newly identified in the past decade and included in the first catalog of rare diseases in China. However, there is still not enough evidence from large samples that can inform the treatment of this disease.

Most IgG4-RD patients respond well to GCs, but the relapse rate is high after GC discontinuation. Although GC maintenance can prevent relapse, its long-term use can lead to side effects and adverse events. During the induction and remission phase, using GCs with IMs can lower the relapse rate in comparison with using GCs alone. However, the role of IMs in maintenance therapy for patients with stable IgG4-RD is still unclear. Therefore, exploring the optimal strategy of maintenance therapy for patients with stable IgG4-RD and the feasibility of tapering GCs are a pressing task that needs solid evidence to inform clinical practices.

The research team led a multicenter clinical study in China, which admitted 146 patients with stable IgG4-RD. Patients with IgG4-RD receiving GC+IM as maintenance treatment with clinically quiescent disease for at least 12 months were randomised 1:1:1 into three groups: group 1: withdraw GC+IM; group 2: withdraw GC but maintain IM; group 3: maintain GC+IM. The study compared the relapse rate, IgG4-RD Responder Index (RI), Physician’s Global Assessment (PGA), and laboratory parameters among the three groups over an 18-month period.

The study showed that in the group withdrawing GC+IM, 52.1% of patients experienced relapse, which was significantly higher than the group withdrawing GC but maintaining IM (14.2%) and the group maintaining GC+IM (12.2%). The group withdrawing GC+IM had significantly higher RI, PGA scores, and increase in serum IgG4 levels than the other two groups. This study confirmed that for patients with long-term stable IgG4-RD, the use of IM with or without low-dose GC are both superior to the withdrawal of IM and GC and help prevent relapse for such patients.

This study is the first randomized controlled trial specifically focusing on maintenance therapies for patients with stable IgG4-RD in the world, filling a gap in this area and providing high-quality evidence that can inform IgG4-RD treatment. The research findings confirm the role of immunosuppressants in preventing disease relapse during maintenance therapy. The research outcome garnered international attention and the team was invited to speak at the American College of Rheumatology 2023 Annual Meeting.

Written by and pictures courtesy of the Department of Rheumatology

Translated by Liu Haiyan

Reviewed by Jiang Nan and Wang Yao