{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,9]],"date-time":"2026-06-09T00:02:51Z","timestamp":1780963371951,"version":"3.54.1"},"reference-count":35,"publisher":"Oxford University Press (OUP)","issue":"1","license":[{"start":{"date-parts":[[2021,8,11]],"date-time":"2021-08-11T00:00:00Z","timestamp":1628640000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/journals\/pages\/open_access\/funder_policies\/chorus\/standard_publication_model"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2022,1,7]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n               <jats:p>A 41-year-old woman with a 14-month history of systemic lupus erythematosus (SLE) presented with headache, aphasia, and agraphia. A laboratory examination revealed mild proteinuria, hypocomplementemia, and elevated anti-double-stranded DNA antibody levels. A cerebrospinal fluid analysis demonstrated elevated protein and interleukin-6 levels. Magnetic resonance imaging (MRI) of the brain identified multiple lesions suggestive of brain edemas and small haemorrhages. She was diagnosed as having neuropsychiatric lupus and lupus nephritis and received remission induction therapy with high-dose corticosteroid and intravenous cyclophosphamide. She achieved a complete remission, and treatment with mycophenolate mofetil (MMF) was initiated 3\u2009months thereafter for remission maintenance. At 13\u2009months after the exacerbation of SLE, she complained of headache and nausea. A gadolinium-enhanced MRI of the brain revealed a low-signal-intensity tumour with marginal ring enhancement of 50\u2009mm in the left frontal lobe. The tumour was excised, and the histological diagnosis was diffuse large B-cell lymphoma with positive Epstein\u2013Barr virus (EBV). MMF was discontinued. Remission induction therapy with rituximab, high-dose methotrexate, procarbazine, and vincristine was administered, and she achieved remission. Previous reports suggest that use of MMF is associated with primary central nervous system (CNS) lymphoma (PCNSL) in patients with lupus nephritis or other autoimmune diseases or in post-transplant patients. Our observation that PCNSL occurred after CNS involvement of SLE suggests that EBV and CNS inflammation arising from SLE might have contributed to the development of PCNSL.<\/jats:p>","DOI":"10.1093\/mrcr\/rxab012","type":"journal-article","created":{"date-parts":[[2021,9,7]],"date-time":"2021-09-07T14:32:41Z","timestamp":1631025161000},"page":"36-40","source":"Crossref","is-referenced-by-count":14,"title":["Primary central nervous system lymphoma in a patient with neuropsychiatric systemic lupus erythematosus receiving mycophenolate mofetil: A case report and literature review"],"prefix":"10.1093","volume":"6","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-2804-485X","authenticated-orcid":false,"given":"Toru","family":"Sakairi","sequence":"first","affiliation":[{"name":"Department of Nephrology and Rheumatology, Gunma University Graduate School of Medicine, Gunma, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Masao","family":"Nakasatomi","sequence":"additional","affiliation":[{"name":"Department of Nephrology and Rheumatology, Gunma University Graduate School of Medicine, Gunma, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mitsuharu","family":"Watanabe","sequence":"additional","affiliation":[{"name":"Department of Nephrology and Rheumatology, Gunma University Graduate School of Medicine, Gunma, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hiroko","family":"Hamatani","sequence":"additional","affiliation":[{"name":"Department of Nephrology and Rheumatology, Gunma University Graduate School of Medicine, Gunma, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hidekazu","family":"Ikeuchi","sequence":"additional","affiliation":[{"name":"Department of Nephrology and Rheumatology, Gunma University Graduate School of Medicine, Gunma, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yoriaki","family":"Kaneko","sequence":"additional","affiliation":[{"name":"Department of Nephrology and Rheumatology, Gunma University Graduate School of Medicine, Gunma, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hiroshi","family":"Handa","sequence":"additional","affiliation":[{"name":"Department of Hematology, Gunma University Graduate School of Medicine, Gunma, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Keiju","family":"Hiromura","sequence":"additional","affiliation":[{"name":"Department of Nephrology and Rheumatology, Gunma University Graduate School of Medicine, Gunma, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2021,8,11]]},"reference":[{"key":"2022011123194277400_R1","doi-asserted-by":"crossref","first-page":"1414","DOI":"10.1038\/bjc.2011.357","article-title":"Age, gender, and racial differences in incidence and survival in primary CNS lymphoma","volume":"105","author":"Villano","year":"2011","journal-title":"Br J Cancer"},{"key":"2022011123194277400_R2","doi-asserted-by":"crossref","first-page":"1287","DOI":"10.4049\/jimmunol.172.2.1287","article-title":"Defective control of latent Epstein-Barr virus infection in systemic lupus erythematosus","volume":"172","author":"Kang","year":"2004","journal-title":"J Immunol"},{"key":"2022011123194277400_R3","doi-asserted-by":"crossref","first-page":"1235","DOI":"10.1136\/annrheumdis-2019-215361","article-title":"Association of Epstein-Barr virus serological reactivation with transitioning to systemic lupus erythematosus in at-risk individuals","volume":"78","author":"Jog","year":"2019","journal-title":"Ann Rheum Dis"},{"key":"2022011123194277400_R4","doi-asserted-by":"crossref","first-page":"699","DOI":"10.1038\/s41588-018-0102-3","article-title":"Transcription factors operate across disease loci, with EBNA2 implicated in autoimmunity","volume":"50","author":"Harley","year":"2018","journal-title":"Nat Genet"},{"key":"2022011123194277400_R5","doi-asserted-by":"crossref","first-page":"45","DOI":"10.1016\/j.leukres.2018.11.004","article-title":"Systemic lupus erythematosus and lymphoma: incidence, pathogenesis and biology","volume":"75","author":"Klein","year":"2018","journal-title":"Leuk Res"},{"key":"2022011123194277400_R6","article-title":"Systemic lupus erythematous and malignancy risk: a meta-analysis","volume":"10","author":"Scheurer","year":"2015","journal-title":"PLoS One"},{"key":"2022011123194277400_R7","doi-asserted-by":"crossref","first-page":"33849","DOI":"10.18632\/oncotarget.5292","article-title":"Primary CNS lymphoproliferative disease, mycophenolate and calcineurin inhibitor usage","volume":"6","author":"Crane","year":"2015","journal-title":"Oncotarget"},{"key":"2022011123194277400_R8","doi-asserted-by":"crossref","first-page":"437","DOI":"10.18632\/oncotarget.26522","article-title":"A single-center experience of post-transplant lymphomas involving the central nervous system with a review of current literature","volume":"10","author":"Velvet","year":"2019","journal-title":"Oncotarget"},{"key":"2022011123194277400_R9","doi-asserted-by":"crossref","first-page":"886","DOI":"10.1177\/0961203306071431","article-title":"Primary lymphoma of CNS, mycophenolate mofetil and lupus","volume":"15","author":"Finelli","year":"2006","journal-title":"Lupus"},{"key":"2022011123194277400_R10","doi-asserted-by":"crossref","first-page":"910","DOI":"10.1191\/0961203303lu2179cr","article-title":"Central nervous system lymphoma associated with mycophenolate mofetil in lupus nephritis","volume":"14","author":"Dasgupta","year":"2005","journal-title":"Lupus"},{"key":"2022011123194277400_R11","doi-asserted-by":"crossref","first-page":"330","DOI":"10.1177\/0961203309347921","article-title":"Diffuse large B-cell lymphoma of the central nervous system in mycophenolate mofetil-treated patients with systemic lupus erythematosus","volume":"19","author":"Tsang","year":"2010","journal-title":"Lupus"},{"key":"2022011123194277400_R12","doi-asserted-by":"crossref","first-page":"144","DOI":"10.3109\/0284186X.2010.504231","article-title":"Use of rituximab in combination with high-dose methotrexate in the treatment of primary central nervous system lymphoma in a mycophenolate mofetil treated patient with lupus nephritis","volume":"50","author":"Lai","year":"2011","journal-title":"Acta Oncol"},{"key":"2022011123194277400_R13","doi-asserted-by":"crossref","first-page":"1452","DOI":"10.1177\/0961203311412413","article-title":"Brain diffuse large B-cell lymphoma in a systemic lupus erythematosus patient treated with immunosuppressive agents including mycophenolate mofetil","volume":"20","author":"Svobodova","year":"2011","journal-title":"Lupus"},{"key":"2022011123194277400_R14","doi-asserted-by":"crossref","first-page":"541","DOI":"10.12809\/hkmj134095","article-title":"Post-transplantation primary central nervous system lymphoma in a patient with systemic lupus erythematosus and prolonged use of immunosuppressant","volume":"20","author":"Tse","year":"2014","journal-title":"Hong Kong Med J"},{"key":"2022011123194277400_R15","doi-asserted-by":"crossref","first-page":"1224","DOI":"10.1177\/0961203317691370","article-title":"Development of primary central nervous system lymphoma in a systemic lupus erythematosus patient after treatment with mycophenolate mofetil and review of the literature","volume":"26","author":"Balci","year":"2017","journal-title":"Lupus"},{"key":"2022011123194277400_R16","article-title":"SLE and non-Hodgkin\u2019s lymphoma: a case series and review of the literature","volume":"2017","author":"Boddu","year":"2017","journal-title":"Case Rep Rheumatol"},{"key":"2022011123194277400_R17","doi-asserted-by":"crossref","DOI":"10.1097\/MD.0000000000011072","article-title":"Primary central nervous system lymphoma in a patient with systemic lupus erythematosus mimicking high-grade glioma a case report and review of literature","volume":"97","author":"Su","year":"2018","journal-title":"Medicine"},{"key":"2022011123194277400_R18","article-title":"Primary central nervous system lymphoma in neuropsychiatric systemic lupus erythematosus: case-based review","author":"Ichikawa","journal-title":"Rheumatol Int"},{"key":"2022011123194277400_R19","doi-asserted-by":"crossref","first-page":"364","DOI":"10.1215\/15228517-2007-004","article-title":"EBV-associated lymphoproliferative disorder of CNS associated with the use of mycophenolate mofetil","volume":"9","author":"O\u2019Neill","year":"2007","journal-title":"Neuro-Oncology"},{"key":"2022011123194277400_R20","doi-asserted-by":"crossref","first-page":"1103","DOI":"10.1097\/NEN.0b013e31818beaea","article-title":"Epstein Barr virus-associated primary CNS lymphomas in elderly patients on immunosuppressive medications","volume":"67","author":"Kleinschmidt-DeMasters","year":"2008","journal-title":"J Neuropathol Exp Neurol"},{"key":"2022011123194277400_R21","doi-asserted-by":"crossref","first-page":"599","DOI":"10.1002\/1529-0131(199904)42:4<599::AID-ANR2>3.0.CO;2-F","article-title":"The American College of Rheumatology nomenclature and case definitions for neuropsychiatric lupus syndromes","volume":"42","year":"1999","journal-title":"Arthritis Rheum"},{"key":"2022011123194277400_R22","volume-title":"Who Classification of Tumours of Haematopoietic and Lymphoid Tissues","author":"Swerdlow","year":"2017"},{"key":"2022011123194277400_R23","doi-asserted-by":"crossref","first-page":"132","DOI":"10.1128\/JCM.37.1.132-136.1999","article-title":"Quantitative analysis of Epstein-Barr virus load by using a real-time PCR assay","volume":"37","author":"Kimura","year":"1999","journal-title":"J Clin Microbiol"},{"key":"2022011123194277400_R24","doi-asserted-by":"crossref","first-page":"138","DOI":"10.1136\/annrheumdis-2012-202099","article-title":"Lymphoma risk in systemic lupus: effects of disease activity versus treatment","volume":"73","author":"Bernatsky","year":"2014","journal-title":"Ann Rheum Dis"},{"key":"2022011123194277400_R25","doi-asserted-by":"crossref","first-page":"225","DOI":"10.1006\/viro.1995.1247","article-title":"Cyclosporin A and FK506 block induction of the Epstein-Barr virus lytic cycle by anti-immunoglobulin","volume":"209","author":"Goldfeld","year":"1995","journal-title":"Virology"},{"key":"2022011123194277400_R26","doi-asserted-by":"crossref","first-page":"3899","DOI":"10.1182\/blood-2009-12-257378","article-title":"An epigenetic chromatin remodeling role for NFATc1 in transcriptional regulation of growth and survival genes in diffuse large B-cell lymphomas","volume":"116","author":"Pham","year":"2010","journal-title":"Blood"},{"key":"2022011123194277400_R27","doi-asserted-by":"crossref","first-page":"569","DOI":"10.1136\/pgmj.63.741.569","article-title":"Systemic lupus erythematosus and primary cerebral lymphoma","volume":"63","author":"Woolf","year":"1987","journal-title":"Postgrad Med J"},{"key":"2022011123194277400_R28","doi-asserted-by":"crossref","first-page":"98","DOI":"10.1017\/S0317167100006405","article-title":"Systemic lupus erythematosus and right leg weakness","volume":"36","author":"Steckley","year":"2009","journal-title":"Can J Neurol Sci"},{"key":"2022011123194277400_R29","doi-asserted-by":"crossref","first-page":"91","DOI":"10.1007\/s11060-004-5676-1","article-title":"Primary CNS lymphoma in myasthenic on long-term azathioprine","volume":"74","author":"Finelli","year":"2005","journal-title":"J Neurooncol"},{"key":"2022011123194277400_R30","doi-asserted-by":"crossref","first-page":"847","DOI":"10.1007\/s00066-014-0670-9","article-title":"Primary EBV-positive Hodgkin\u2019s lymphoma of the CNS under azathioprine treatment","volume":"190","author":"Henkenberens","year":"2014","journal-title":"Strahlenther Onkol"},{"key":"2022011123194277400_R31","doi-asserted-by":"crossref","first-page":"121","DOI":"10.2176\/nmccrj.cr.2019-0241","article-title":"Primary central nervous system methotrexate-associated lymphoproliferative disorder in a patient with rheumatoid arthritis: case report and review of literature","volume":"7","author":"Uneda","year":"2020","journal-title":"NMC Case Rep J"},{"key":"2022011123194277400_R32","doi-asserted-by":"crossref","first-page":"1797","DOI":"10.1038\/leu.2011.169","article-title":"Modern concepts in the biology, diagnosis, differential diagnosis and treatment of primary central nervous system lymphoma","volume":"25","author":"Deckert","year":"2011","journal-title":"Leukemia"},{"key":"2022011123194277400_R33","first-page":"92","article-title":"Multiple sclerosis preceding CNS lymphoma: a case report","volume":"16","author":"Yang","year":"2007","journal-title":"Acta Neurol Taiwan"},{"key":"2022011123194277400_R34","doi-asserted-by":"crossref","first-page":"432","DOI":"10.1080\/13550280260422730","article-title":"Epstein-Barr virus DNA load in cerebrospinal fluid and plasma of patients with AIDS-related lymphoma","volume":"8","author":"Bossolasco","year":"2002","journal-title":"J Neurovirol"},{"key":"2022011123194277400_R35","doi-asserted-by":"crossref","first-page":"495","DOI":"10.1007\/s12185-011-0951-3","article-title":"Discrepancy in EBV-DNA load between peripheral blood and cerebrospinal fluid in a patient with isolated CNS post-transplant lymphoproliferative disorder","volume":"94","author":"Shimizu","year":"2011","journal-title":"Int J Hematol"}],"container-title":["Modern Rheumatology Case Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/academic.oup.com\/mrcr\/advance-article-pdf\/doi\/10.1093\/mrcr\/rxab012\/41745803\/rxab012.pdf","content-type":"application\/pdf","content-version":"am","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/mrcr\/article-pdf\/6\/1\/36\/42106701\/rxab012.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/mrcr\/article-pdf\/6\/1\/36\/42106701\/rxab012.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2022,1,19]],"date-time":"2022-01-19T02:47:00Z","timestamp":1642560420000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/mrcr\/article\/6\/1\/36-40\/6348229"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,8,11]]},"references-count":35,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2021,8,11]]},"published-print":{"date-parts":[[2022,1,7]]}},"URL":"https:\/\/doi.org\/10.1093\/mrcr\/rxab012","relation":{},"ISSN":["2472-5625"],"issn-type":[{"value":"2472-5625","type":"electronic"}],"subject":[],"published-other":{"date-parts":[[2022,1,1]]},"published":{"date-parts":[[2021,8,11]]}}}