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Comparison of the efficacy and safety of leflunomide versus mycophenolate mofetil in treating IgG4-related disease: a retrospective cohort study

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Abstract

Objective

Combination therapy of glucocorticoids (GCs) plus leflunomide (LEF) and GCs plus mycophenolate mofetil (MMF) was reported to have good efficacy and safety in the management of IgG4-RD. However, studies comparing the efficacy and safety of these two combination therapies were unavailable. Herein, this study aimed to compare the efficacy and safety of GCs plus LEF and GCs plus MMF in treating IgG4-RD.

Methods

This study included 130 newly diagnosed IgG4-RD patients who received the therapy of GCs plus LEF (group I) and GCs plus MMF (group II). Clinical data at baseline and after treatment, treatment response, relapse rate, and adverse effects were recorded and analyzed.

Results

Patients in both groups responded well to the treatment in the 1st-month follow-up, and 100% of patients achieved treatment response. However, at the 6th and 12th-month follow-up, the total response rate of group II was higher than that in group I (75.6 vs. 53.7%, p = 0.038 and 85.4% vs. 61.0%, p = 0.013, respectively). In addition, the duration of disease remission in group II was longer than that in group I (9 (6–9) vs. 6 (6–6) months, p = 0.014). Moreover, more patients in group I had adverse effects compared with group II (36.6 vs. 7.3%, p < 0.01); and the most common adverse events of LEF were rash (12.2%) and elevation of liver enzymes (9.8%).

Conclusion

The combination therapy of GCs plus low-dose MMF had better efficacy and safety in the management of IgG4-RD compared with the therapy of GCs plus LEF.

Key points

• This is the first study comparing the efficacy and safety of GCs plus LEF/MMF against IgG4-RD.

• GCs plus MMF had better efficacy and safety in the management of IgG4-RD.

• This study provides physicians with decision-making advice in choosing a treatment strategy for IgG4-RD.

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Data Availability

The datasets used and/or analysed during the current study are available from the corresponding author on reasonable request.

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Funding

This work was supported by the National Natural Science Foundation of China (No.81971545, No.82071839), Dongcheng District Outstanding Talent Nurturing Program (2022-dchrcpyzz-67) and the CAMS Innovation Fund for Medical Sciences (CIFMS): 2020-I2M-C&T-A-002 and CIFMS 2021–1-I2M-003. National High Level Hospital Clinical Research Funding (2022-PUMCH-A-005).

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Author notes

  1. Yingying Chen and Rongli Li contributed equally to this paper.

Authors and Affiliations

  1. Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China

    Yingying Chen, Rongli Li, Xuan Luo, Jieqiong Li, Zheng Liu, Yu Peng, Hui Lu, Linyi Peng, Jiaxin Zhou, Yan Zhao, Xiaofeng Zeng, Yunyun Fei & Wen Zhang

  2. National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), Ministry of Science & Technology, Beijing, China

    Yingying Chen, Rongli Li, Xuan Luo, Jieqiong Li, Zheng Liu, Yu Peng, Hui Lu, Linyi Peng, Jiaxin Zhou, Yan Zhao, Xiaofeng Zeng, Yunyun Fei & Wen Zhang

  3. State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital (PUMCH), Beijing, China

    Yingying Chen, Rongli Li, Xuan Luo, Jieqiong Li, Zheng Liu, Yu Peng, Hui Lu, Linyi Peng, Jiaxin Zhou, Yan Zhao, Xiaofeng Zeng, Yunyun Fei & Wen Zhang

  4. Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, 100730, China

    Yingying Chen, Rongli Li, Xuan Luo, Jieqiong Li, Zheng Liu, Yu Peng, Hui Lu, Linyi Peng, Jiaxin Zhou, Yan Zhao, Xiaofeng Zeng, Yunyun Fei & Wen Zhang

  5. Department of Biostatistics, Bioinformatics, and Biomathematics, Georgetown University, Washington, DC, USA

    Tianmin Wu

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  1. Yingying Chen
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  2. Rongli Li
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  7. Yu Peng
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  11. Yan Zhao
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Corresponding authors

Correspondence to Yunyun Fei or Wen Zhang.

Ethics declarations

Ethics approval and consent to participate

The study was approved by the Ethics Committee of the Peking Union Medical College Hospital (Number: S-442) and was conducted in accordance with the Helsinki Declaration. Written informed consent was obtained from all patients included in this study.

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Chen, Y., Li, R., Luo, X. et al. Comparison of the efficacy and safety of leflunomide versus mycophenolate mofetil in treating IgG4-related disease: a retrospective cohort study. Clin Rheumatol 42, 1839–1846 (2023). https://doi.org/10.1007/s10067-023-06528-5

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  • DOI: https://doi.org/10.1007/s10067-023-06528-5

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