The Complexities of Neuropsychiatric Lupus: Pathogenesis, Diagnosis, and Management
Ramesh T V1, Gopi Ayyaswamy2, Zioni Sangeetha3, Vinod Kumar P 4*
Journal of Angiotherapy 6(1) 1-5 https://doi.org/10.25163/angiotherapy.6162186290707122
Submitted: 29 November 2021 Revised: 14 December 2021 Published: 07 January 2022
Abstract
Background: Systemic Lupus Erythematosus (SLE) is a chronic autoimmune disease with widespread systemic involvement, including neurological manifestations that affect 10 to 80% of patients. Neurological symptoms in SLE can be diverse, ranging from headaches to severe conditions like CNS vasculitis. Methodology: We present a case of a 32-year-old female with a two-year history of SLE who had been non-compliant with her treatment for six months. She presented with new-onset symptoms, including low-grade fever, persistent headache, significant weight loss, tinnitus with hearing loss, and polyarthralgia. Laboratory tests and MRI were used for diagnosis. Results: Laboratory findings revealed elevated anti-dsDNA antibodies, decreased complement levels (C3 and C4), and increased serum Lactate Dehydrogenase (LDH). MRI of the brain showed diffuse T2 FLAIR hyperintensities, indicative of CNS vasculitis. The patient was diagnosed with CNS vasculitis secondary to SLE. Conclusion: The patient was treated with Mycophenolate Mofetil 500 mg twice daily, Hydroxychloroquine 200 mg nightly, and Methylprednisolone 8 mg daily. Significant improvement and resolution of symptoms were achieved within two weeks. This case highlights the importance of early diagnosis and aggressive treatment in managing SLE-related CNS vasculitis, emphasizing the need for adherence to treatment and regular follow-up to prevent disease progression.
Keywords: SLE, CNS vasculitis, anti-dsDNA, Mycophenolate Mofetil, Hydroxychloroquine, Methylprednisolone
References
Abdel-Nasser, A. M., Ghaleb, R. M., Mahmoud, J. A., Khairy, W., & Mahmoud, R. M. (2008). Association of anti-ribosomal P protein antibodies with neuropsychiatric and other manifestations of systemic lupus erythematosus. Clinical Rheumatology, 27(11), 1377–1385. https://doi.org/10.1007/s10067-008-0921-1
Arinuma, Y., Kikuchi, H., & Hirohata, S. (2019). Anti-ribosomal P protein antibodies influence mortality of patients with diffuse psychiatric/neuropsychological syndromes in systemic lupus erythematosus involving a severe form of the disease. Modern Rheumatology, 29(4), 612–618. https://doi.org/10.1080/14397595.2018.1508801
Aschman, T., Schaffer, S., Biniaris Georgallis, S. I., Triantafyllopoulou, A., Staeheli, P., & Voll, R. E. (2021). Interferon lambda regulates cellular and humoral immunity in pristane-induced lupus. International Journal of Molecular Sciences, 22(21), 11747. https://doi.org/10.3390/ijms222111747
Bertsias, G. K., & Boumpas, D. T. (2010). Pathogenesis, diagnosis, and management of neuropsychiatric SLE manifestations. Nature Reviews Rheumatology, 6(6), 358–367. https://doi.org/10.1038/nrrheum.2010.62
Bertsias, G. K., Ioannidis, J. P., Aringer, M., Bollen, E., Bombardieri, S., Bruce, I. N., et al. (2010). EULAR recommendations for the management of systemic lupus erythematosus with neuropsychiatric manifestations: Report of a task force of the EULAR standing committee for clinical affairs. Annals of the Rheumatic Diseases, 69(12), 2074–2082. https://doi.org/10.1136/ard.2010.130476
Bialas, A. R., Presumey, J., Das, A., van der Poel, C. E., Lapchak, P. H., Mesin, L., et al. (2020). Retraction Note: Microglia-dependent synapse loss in type I interferon-mediated lupus. Nature, 578(7793), 177. https://doi.org/10.1038/s41586-020-1949-x
Bravo-Zehnder, M., Toledo, E. M., Segovia-Miranda, F., Serrano, F. G., Benito, M. J., Metz, C., et al. (2015). Anti-ribosomal P protein autoantibodies from patients with neuropsychiatric lupus impair memory in mice. Arthritis & Rheumatology, 67(1), 204–214. https://doi.org/10.1002/art.38900
Cieslik, P., Semik-Grabarczyk, E., Hrycek, A., & Holecki, M. (2022). The impact of anti-endothelial cell antibodies (AECAs) on the development of blood vessel damage in patients with systemic lupus erythematosus: The preliminary study. Rheumatology International, 42(5), 791–801. https://doi.org/10.1007/s00296-022-05104-5
Cohen, D., Rijnink, E. C., Nabuurs, R. J., Steup-Beekman, G. M., Versluis, M. J., Emmer, B. J., et al. (2017). Brain histopathology in patients with systemic lupus erythematosus: Identification of lesions associated with clinical neuropsychiatric lupus syndromes and the role of complement. Rheumatology, 56(1), 77–86. https://doi.org/10.1093/rheumatology/kew341
Delunardo, F., Soldati, D., Bellisario, V., Berry, A., Camerini, S., Crescenzi, M., et al. (2016). Anti-GAPDH autoantibodies as a pathogenic determinant and potential biomarker of neuropsychiatric diseases. Arthritis & Rheumatology, 68(11), 2708–2716. https://doi.org/10.1002/art.39750
Donnellan, C., Cohen, H., & Werring, D. J. (2021). Cognitive dysfunction and associated neuroimaging biomarkers in antiphospholipid syndrome: A systematic review. Rheumatology, 61(1), 24–41. https://doi.org/10.1093/rheumatology/keab452
Eber, T., Chapman, J., & Shoenfeld, Y. (2005). Anti-ribosomal P-protein and its role in psychiatric manifestations of systemic lupus erythematosus: Myth or reality? Lupus, 14(8), 571–575. https://doi.org/10.1191/0961203305lu2150rr
Everett, C. M., Graves, T. D., Lad, S., Jäger, H. R., Thom, M., Isenberg, D. A., & Hanna, M. G. (2008). Aggressive CNS lupus vasculitis in the absence of systemic disease activity. Rheumatology, 47(1), 107–109. https://doi.org/10.1093/rheumatology/kem264
Fanali, G., di Masi, A., Trezza, V., Marino, M., Fasano, M., & Ascenzi, P. (2012). Human serum albumin: From bench to bedside. Molecular Aspects of Medicine, 33(3), 209–290. https://doi.org/10.1016/j.mam.2011.12.002
Fleetwood, T., Cantello, R., & Comi, C. (2018). Antiphospholipid syndrome and the neurologist: From pathogenesis to therapy. Frontiers in Neurology, 9, 1001. https://doi.org/10.3389/fneur.2018.01001
Gelb, S., Stock, A. D., Anzi, S., Putterman, C., & Ben-Zvi, A. (2018). Mechanisms of neuropsychiatric lupus: The relative roles of the blood-cerebrospinal fluid barrier versus blood-brain barrier. Journal of Autoimmunity, 91, 34–44. https://doi.org/10.1016/j.jaut.2018.03.001
Kakati, S., Barman, B., Ahmed, S. U., & Hussain, M. (2017). Neurological manifestations in systemic lupus erythematosus: A single centre study from North East India. Journal of Clinical and Diagnostic Research, 11(1), OC05–OC09. https://doi.org/10.7860/JCDR/2017/23773.9280
Rowshani, A. T., Remans, P., Rozemuller, A., et al. (2005). Cerebral vasculitis as a primary manifestation of systemic lupus erythematosus. Annals of the Rheumatic Diseases, 64, 784–786. https://doi.org/10.1136/ard.2004.026542
View Dimensions
View Altmetric
Save
Citation
View
Share