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Primary central nervous system lymphoma in neuropsychiatric systemic lupus erythematosus: case-based review

Rheumatology International volume 41, pages 1009–1017 (2021)Cite this article

Abstract

Primary central nervous system lymphoma (PCNSL) sometimes occurs in immune-compromised hosts or patients with autoimmune diseases. Some cohort studies have previously reported an increased risk of non-Hodgkin’s lymphoma in systemic lupus erythematosus (SLE), while some cases of PCNSL in patients with SLE were reported. We present the case of PCNSL which developed in a patient with the active phase of neuropsychiatric SLE (NPSLE). Furthermore, we reviewed published English articles to confirm the characteristics of PCNSL related to SLE. To our knowledge, this is the first report of PCNSL occurring in NPSLE. Histology demonstrated B-cell lymphoma with a positive Epstein–Barr virus-encoded RNA. This patient recovered following surgical resection of the lymphoma, whole brain radiation therapy, intravenous infusion of rituximab (RTX), and administration of belimumab after RTX. Given the series of reviews, our report suggests that the persistence of damage in the central nervous system (CNS) and long-term exposure to immunosuppressants may impact oncogenic immune responses within the CNS, leading to PCNSL development.

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Acknowledgements

We wish to thank the members of the Department of Neurosurgery for their efforts in providing medical care. We would like to thank Editage (www.editage.com) for English editing.

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Affiliations

  1. Department of Medicine (Neurology and Rheumatology), Shinshu University School of Medicine, 3-1-1 Asahi, Matsumoto, 390-8621, Japan

    Takanori Ichikawa, Yasuhiro Shimojima, Dai Kishida & Yoshiki Sekijima

  2. Department of Radiology, Shinshu University School of Medicine, 3-1-1 Asahi, Matsumoto, 390-8621, Japan

    Tomoki Kaneko

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  1. Takanori Ichikawa
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  2. Yasuhiro Shimojima
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Contributions

The diagnosis and medical care were performed by T-I, Y-S, D-K, and Y-S. Radiological evaluation and diagnostic support were provided by T-K. All authors conceived and made the design of this case report. T-I and Y-S prepared the draft of this manuscript. Y-S and Y-S revised the manuscript. All authors approved the final manuscript as submitted and agreed to be responsible for all aspects of this report.

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Correspondence to Yasuhiro Shimojima.

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Ichikawa, T., Shimojima, Y., Kishida, D. et al. Primary central nervous system lymphoma in neuropsychiatric systemic lupus erythematosus: case-based review. Rheumatol Int 41, 1009–1017 (2021). https://doi.org/10.1007/s00296-020-04569-6

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  • DOI: https://doi.org/10.1007/s00296-020-04569-6

Keywords

  • Primary central nervous system lymphoma
  • Systemic lupus erythematosus
  • Neuropsychiatric systemic lupus erythematosus
  • Epstein–Barr virus
  • Immunosuppressant