Withdrawal of Immunosuppressant and Low-dose Steroids in IgG4-RD Patients with Stable Disease (WInS IgG4-RD): An Investigator-initiated, Multi-center, Open-label, Randomized Controlled Trial

Linyi Peng¹, Yuxue nie¹, Jiaxin Zhou¹, Lijun Wu², Fang Wang³, Xiaomei Chen⁴, Jieqiong Li¹, Yu Peng¹, Hui Lu¹, Lidan Zhao¹, Mengtao Li¹, Yan Zhao¹, Xiaofeng Zeng⁵, Yunyun Fei¹ and Wen Zhang¹, ¹Peking Union Medical College Hospital，Department of Rheumatology and Clinical Immunology, National Clinical Research Center for Dermatologic and Immunologic Diseases, State Key Laboratory of Complex Severe and Rare Diseases, Beijing, China, ²Xinjiang Uygur Autonomous Region People's Hospital, XinJiang, China, ³Beijing Hospital, Beijing, China, ⁴Xinjiang Uygur Autonomous Region People's Hospital, Wulumuqi, China, ⁵Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, Beijing, China

Meeting: ACR Convergence 2023

Date of first publication: October 24, 2023

Keywords: Disease-Modifying Antirheumatic Drugs (Dmards), glucocorticoids, IgG4 Related Disease, Late-Breaking 2023, Randomized Trial, Therapy, alternative

Session Information

Date: Wednesday, November 15, 2023

Title: Late-Breaking Abstract Session

Session Type: Late-Breaking Abstract Session

Session Time: 7:30AM-9:00AM

Background/Purpose: IgG4-related disease (IgG4-RD) is a fibroinflammatory disease. Remission induction treatment with glucocorticoid (GC) is usually effective, but its tendency of relapse makes the strategy for maintenance treatment a challenge. TheWInS IgG4-RD (withdraw immunosuppressants and steroid in stable IgG4-RD) trial tested whether discontinuation of GC and immunosuppressive agent (IM) were feasible in stable IgG4-RD.

Methods: The WInS IgG4-RD trial was a multicentre, open-label, randomised controlled trial. IgG4-RD patients receiving GC+IM as maintenance treatment with clinically quiescent disease for at least 12 months were randomised (1:1:1) into three groups: (1) Group 1: withdraw GC+IM; (2) Group 2: withdraw GC but maintain IM; (3) Group 3: maintain GC+IM. The primary endpoint was the relapse rate of disease within 18 months. The secondary endpoints include the changes of responder index (RI), physician’s global assessment (PGA), serum IgG4 and IgG, as well as adverse events.

Results: One hundred and forty-six patients were randomised, with 48 patients in Group 1, and 49 patients in Group 2 and Group 3 respectively. Within the 18-month follow-up period, disease relapse occurred in 25/48 (52.1%) patients in Group 1 versus 7/49 (14.2%) in Group 2 and 6/49 (12.2%) in Group 3 (p< 0.001). The changes in RI and PGA were significantly higher in Group 1 than Group 2 (p< 0.001) or Group 3 (p< 0.001).

Conclusion: The maintenance of IMs, with or without low-dose GC, was found to be superior to withdraw GC+IM in preventing relapse for long-time stable IgG4-RD.

Flowchart of WInS IgG4-RD study

The occurrence of relapse during follow-up is presented as a Kaplan-Meier curve of cumulative probability (Figure 2), where there were significant differences among the three groups by both ITT and PP analysis (both overall p<0.001).

Changes of IgG4(a), IgG (b), IgG4-RD RI (3c), PGA (figure 3d) at baseline of the study and the endpoints by intention-to-treat analysis. RI, Responder index; PGA, physician’s global assessment.

Disclosures: L. Peng: None; Y. nie: None; J. Zhou: None; L. Wu: None; F. Wang: None; X. Chen: None; J. Li: None; Y. Peng: None; H. Lu: None; L. Zhao: None; M. Li: None; Y. Zhao: None; X. Zeng: None; Y. Fei: None; W. Zhang: None.

To cite this abstract in AMA style:

Peng L, nie Y, Zhou J, Wu L, Wang F, Chen X, Li J, Peng Y, Lu H, Zhao L, Li M, Zhao Y, Zeng X, Fei Y, Zhang W. Withdrawal of Immunosuppressant and Low-dose Steroids in IgG4-RD Patients with Stable Disease (WInS IgG4-RD): An Investigator-initiated, Multi-center, Open-label, Randomized Controlled Trial [abstract]. Arthritis Rheumatol. 2023; 75 (suppl 9). https://acrabstracts.org/abstract/withdrawal-of-immunosuppressant-and-low-dose-steroids-in-igg4-rd-patients-with-stable-disease-wins-igg4-rd-an-investigator-initiated-multi-center-open-label-randomized-controlled-trial/. Accessed .

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