Treatment strategies for relapsing IgG4-RD: twelve-year experience from a prospective cohort

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ID: 324614
2026
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Abstract
OBJECTIVES: Relapse is common in IgG4-related disease (IgG4-RD). While previous studies focus on treatments for active IgG4-RD, data on the management of relapsing IgG4-RD remains limited. We aimed to investigate the treatment strategies for relapsing IgG4-RD. METHODS: This study analyzed data prospectively collected from an IgG4-RD cohort, including patients who experienced their first relapse and were followed for over 12 months. We compared the outcome among three groups: (1) Intensifying glucocorticoids (GC) only; (2) Intensifying immunosuppressants (IM) only; (3) Intensifying both GC and IM. The primary outcome was recurrent relapses. A decision tree model was developed to predict recurrent relapses. RESULTS: A total of 259 patients with IgG4-RD was included, with a male predominance (61.7%) and a median age of 54.0 years. Patients who intensified both GC and immunosuppressants (IM) (adjusted p 0.0002, HR 0.29, 95% CI: 0.16-0.52) experienced less relapses compared to those who intensified IM alone, while those who only intensified GC had a tendency to experience less relapses (adjusted p 0.0823, HR 0.51, 95% CI: 0.26-1.03). These differences were more pronounced in the subgroup with internal organ involvement. The decision tree indicated that those with an RI decrease to ≤ 2 and an IgG4 decrease ≥25% after treatment were more likely to stay free from recurrent relapses, with an AUC of 0.728 in the testing set. CONCLUSION: When treating relapse in IgG4-RD, GC-based treatment was associated with a lower hazard of recurrent relapse compared with only adjusting IMs, especially for those with internal organ involvement.
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Authors Yuxue Nie, Jialei Zhang, Jialing Jiang, Hui Lu, Xinli Yang, Jingna Li, Nianyi Zhang, Jie Meng, Lidan Zhao, Li Zhang, Jiaxin Zhou, Yunyun Fei, Yan Zhao, Xiaofeng Zeng, Mengtao Li, Linyi Peng, Wen Zhang
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Journal Lara D. Veeken
Year 2026
DOI
10.1093/rheumatology/keag401
URL
https://doi.org/10.1093/rheumatology/keag401
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