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An overlapping case of IgG4-related disease and systemic lupus erythematosus treated with belimumab: a case-based review

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Abstract

IgG4-related disease (IgG4-RD) is a systemic condition in which IgG4+ plasma cell infiltration and fibrosis cause organ swelling and lead to diverse clinical manifestations. Although IgG4-RD typically responds to glucocorticoids (GCs), relapse during tapering occurs and an early GC-sparing approach might therefore be beneficial. Systemic lupus erythematosus (SLE) is a chronic inflammatory disease with multiple symptoms that is also treated with GCs as a first-line therapy. Recently, belimumab, a recombinant human IgG-1λ monoclonal antibody that inhibits B-cell activating factor, was approved, but reports of use for IgG4-RD are scarce. Here, we present a rare case of IgG4-RD complicated with SLE which was successfully treated with belimumab. A 67-year-old man was diagnosed with IgG4-RD based on a high serum IgG4 level and histopathological findings. Furthermore, he had pericardial effusion on echocardiography, and laboratory tests revealed thrombocytopenia, autoimmune hemolysis, positive anti-nuclear antibodies, positive anti-DNA antibodies, and hypocomplementemia. These data led to an SLE diagnosis. Treatment was started with prednisolone at 40 mg/day, plus hydroxychloroquine, which initially improved both the SLE and IgG4-RD symptoms. During the GC tapering, belimumab was added and clinical symptoms resolved completely. Our case and the literature review summarize reported rare overlapping cases of IgG4-RD and SLE and suggest that belimumab is a promising candidate for the treatment of IgG4-RD.

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Acknowledgements

The authors would like to thank Dr. Bryan J. Mathis of the University of Tsukuba Hospital International Medical Center for language revision.

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  1. Megumi Iwamoto and Hiromitsu Asashima contributed equally.

Authors and Affiliations

  1. Department of Rheumatology, Institute of Medicine, University of Tsukuba, 1-1-1 Tennodai, Tsukuba, Ibaraki, 305-8575, Japan

    Megumi Iwamoto, Hiromitsu Asashima, Toshiki Sugita, Fumina Kawashima, Naoki Sugita, Akiyoshi Rai, Yuki Kuroda, Akira Kawashima, Daiki Tabuchi, Satoshi Akao, Ryota Sato, Taihei Nishiyama, Hirofumi Toko, Fumika Honda, Ayako Ohyama, Ayako Kitada, Saori Abe, Haruka Miki, Shinya Hagiwara, Yuya Kondo, Hiroto Tsuboi & Isao Matsumoto

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Contributions

MI performed the literature review and wrote the manuscript. HA and IM supervised the process from drafting the paper to submission. HA, TS, and FK were involved in the clinical management. All the authors have contributed to data collection, reviewed the manuscripts, and take full responsibility for the integrity and accuracy of all aspects of the work.

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Correspondence to Isao Matsumoto.

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Iwamoto, M., Asashima, H., Sugita, T. et al. An overlapping case of IgG4-related disease and systemic lupus erythematosus treated with belimumab: a case-based review. Rheumatol Int 44, 549–556 (2024). https://doi.org/10.1007/s00296-023-05510-3

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