Generic placeholder image

Current Rheumatology Reviews


ISSN (Print): 1573-3971
ISSN (Online): 1875-6360

Case Report

Neuropsychiatric Systemic Lupus Erythematosus and Posterior Reversible Encephalopathy Syndrome in a Young Woman: A Case Report

Author(s): Yasser Mohammed Bawazir* and Neda'a Wasim Anshasi

Volume 19, Issue 4, 2023

Published on: 19 May, 2023

Page: [508 - 514] Pages: 7

DOI: 10.2174/1573397119666230420111517

Price: $65


Introduction: Systemic Lupus Erythematosus (SLE) is a chronic autoimmune disease with variable clinical presentation, including neuropsychiatric manifestations. It has a different diagnostic approach and several different therapeutic options.

Case Report: We describe a case of a young woman who first presented with arthritis, serositis, and pancreatitis, and was treated with mycophenolate mofetil initially. The patient presented with neurological symptoms suggestive of neuropsychiatric manifestations three weeks later, confirmed by Brain Magnetic Resonance Imaging (MRI). The treatment was changed to cyclophosphamide; however, the day after the infusion, she developed status epilepticus and was admitted to the intensive care unit. Repeated brain MRI revealed Posterior Reversible Encephalopathy Syndrome (PRES). Cyclophosphamide was discontinued and rituximab was initiated. The patient’s neurological manifestations improved, and she was discharged after 25 days of use.

Conclusion: Immunosuppressive agents, such as cyclophosphamide have been described as a potential risk factor for PRES; however, it is not clear from the available literature whether cyclophosphamide therapy is just a marker of more severe SLE or a true risk factor for PRES.

Keywords: SLE, PRESS, neuropsychiatric lupus, and cyclophosphamide, MRI, autoimmune disease.

Graphical Abstract
Aringer M, Johnson SR. Systemic Lupus erythematosus classification and diagnosis. Rheum Dis Clin North Am 2021; 47(3): 501-11.
[] [PMID: 34215376]
Carrión-Barberà I, Salman-Monte TC, Vílchez-Oya F, Monfort J. Neuropsychiatric involvement in systemic lupus erythematosus: A review. Autoimmun Rev 2021; 20(4): 102780.
[] [PMID: 33609799]
The American College of Rheumatology nomenclature and case definitions for neuropsychiatric lupus syndromes. Arthritis Rheum 1999; 42(4): 599-608.
[<599:AID-ANR2>3.0.CO;2-F] [PMID: 10211873]
Ainiala H, Hietaharju A, Loukkola J, et al. Validity of the new American College of Rheumatology criteria for neuropsychiatric lupus syndromes: A population‐based evaluation. Arthritis Rheum 2001; 45(5): 419-23.
[<419:AID-ART360>3.0.CO;2-X] [PMID: 11642640]
Barile-Fabris L, Ariza-Andraca R, Olguín-Ortega L, et al. Controlled clinical trial of IV cyclophosphamide versus IV methylprednisolone in severe neurological manifestations in systemic lupus erythematosus. Ann Rheum Dis 2005; 64(4): 620-5.
[] [PMID: 15769918]
Fanouriakis A, Kostopoulou M, Alunno A, et al. 2019 update of the EULAR recommendations for the management of systemic lupus erythematosus. Ann Rheum Dis 2019; 78(6): 736-45.
[] [PMID: 30926722]
Manadan A, Kambhatla S, Gauto-Mariotti E, Okoli C, Block JA. Rheumatic diseases associated with posterior reversible encephalopathy syndrome. J Clin Rheumatol 2021; 27(8): e391-4.
[] [PMID: 32604240]
Zekić T, Benić MS, Antulov R, Antončić I, Novak S. The multifactorial origin of posterior reversible encephalopathy syndrome in cyclophosphamide-treated lupus patients. Rheumatol Int 2017; 37(12): 2105-14.
[] [PMID: 29043491]
Pons-Estel GJ, Alarcón GS, Scofield L, Reinlib L, Cooper GS. Understanding the epidemiology and progression of systemic Lupus erythematosus. Seminars in arthritis and rheumatism
Alves SC, Fasano S, Isenberg DA. Autoimmune gastrointestinal complications in patients with systemic lupus erythematosus: Case series and literature review. Lupus 2016; 25(14): 1509-19.
[] [PMID: 27329649]
Makol A, Petri M. Pancreatitis in systemic lupus erythematosus: Frequency and associated factors - a review of the Hopkins Lupus Cohort. J Rheumatol 2010; 37(2): 341-5.
[] [PMID: 20032096]
Tian XP, Zhang X. Gastrointestinal involvement in systemic lupus erythematosus: Insight into pathogenesis, diagnosis and treatment. World J Gastroenterol 2010; 16(24): 2971-7.
[] [PMID: 20572299]
Jeltsch-David H, Muller S. Neuropsychiatric systemic lupus erythematosus: Pathogenesis and biomarkers. Nat Rev Neurol 2014; 10(10): 579-96.
[] [PMID: 25201240]
Hanly JG, Urowitz MB, Sanchez-Guerrero J, et al. Neuropsychiatric events at the time of diagnosis of systemic lupus erythematosus: An international inception cohort study. Arthritis Rheum 2007; 56(1): 265-73.
[] [PMID: 17195230]
Hanly JG, Kozora E, Beyea SD, Birnbaum J. Nervous system disease in systemic Lupus erythematosus: Current status and future directions. Arthritis Rheumatol 2019; 71(1): 33-42.
[] [PMID: 29927108]
Lai CC, Chen WS, Chang YS, et al. Clinical features and outcomes of posterior reversible encephalopathy syndrome in patients with systemic lupus erythematosus. Arthritis Care Res 2013; 65(11): 1766-74.
[] [PMID: 23687067]
Canney M, Kelly D, Clarkson M. Posterior reversible encephalopathy syndrome in end-stage kidney disease: not strictly posterior or reversible. Am J Nephrol 2015; 41(3): 177-82.
[] [PMID: 25871433]
Miller JB, Suchdev K, Jayaprakash N, et al. New developments in hypertensive encephalopathy. Curr Hypertens Rep 2018; 20(2): 13.
[] [PMID: 29480370]
Abenza-Abildua MJ, Fuentes B, Diaz D, et al. Cyclophosphamide-induced reversible posterior leukoencephalopathy syndrome. Case Rep 2009; 2009: bcr0720080467.
How J, Blattner M, Fowler S, Wang-Gillam A, Schindler SE. Chemotherapy-associated posterior reversible encephalopathy syndrome: A case report and review of the literature. Neurologist 2016; 21(6): 112-7.
[] [PMID: 27801773]
Lee CH, Lee YM, Ahn SH, Ryu DW, Song JH, Lee MS. Cyclophosphamide-induced posterior reversible encephalopathy syndrome in a patient with lupus nephritis. J Rheum Dis 2013; 20(2): 103-7.
Jabrane M, Lahcen ZA, Fadili W, Laouad I. A case of PRES in an active lupus nephritis patient after treatment of corticosteroid and cyclophosphamide. Rheumatol Int 2015; 35(5): 935-8.
[] [PMID: 25387825]
Buttar C, Lakhdar S, Castillo F, Abrudescu A. Cyclophosphamide induced posterior reversible encephalopathy syndrome (PRES) in a patient with lupus nephritis. Clin Med Rev Case Rep 2021; 8: 354.
Tabot MKT, Ababio PA, Waldron S, Rougui L, Mehari A. A rare case of cyclophosphamide-induced posterior reversible encephalopathy syndrome in a patient with acute lupus nephritis flare. Cureus 2023; 15(1): 34372.

Rights & Permissions Print Cite
© 2024 Bentham Science Publishers | Privacy Policy