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Systemic Lupus Erythematosus
Systemic Lupus Erythematosus
Surjith Vattoth, MD, FRCR
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Abbreviations

                • Systemic lupus erythematosus (SLE)
                • Neuropsychiatric SLE (NPSLE)

              • Definitions

                • Primary NPSLE: If resulting directly from immune-mediated injury
                • Secondary NPSLE: If related to treatment, infections, metabolic disturbances, or other systemic manifestations not related to SLE; ~ 60%

              IMAGING

              • General Features

                • CT Findings

                  • MR Findings

                    • Angiographic Findings

                      • Nuclear Medicine Findings

                        • Imaging Recommendations

                          DIFFERENTIAL DIAGNOSIS

                            PATHOLOGY

                            • General Features

                              • Staging, Grading, & Classification

                                • Gross Pathologic & Surgical Features

                                  • Microscopic Features

                                    CLINICAL ISSUES

                                    • Presentation

                                      • Demographics

                                        • Natural History & Prognosis

                                          • Treatment

                                            DIAGNOSTIC CHECKLIST

                                            • Consider

                                              • Image Interpretation Pearls

                                                Selected References

                                                1. John TJ et al: SLE pachymeningitis and multiple cranial nerve palsies: a case report and review of the literature. Lupus. 28(9):1154-7, 2019
                                                2. Mvambo N et al: Clinical, neuroimaging and immunological phenotype of South African neuropsychiatric systemic lupus erythematosus patients. Lupus. 28(5):685-94, 2019
                                                3. Yeoh H et al: Relationship between cerebral microbleeds and white matter MR hyperintensities in systemic lupus erythematosus: a retrospective observational study. Neuroradiology. 61(3):265-74, 2019
                                                4. McGlasson S et al: Neurological Disease in Lupus: Toward a Personalized Medicine Approach. Front Immunol. 9:1146, 2018
                                                5. Vivaldo JF et al: Definition of NPSLE: Does the ACR Nomenclature Still Hold? Front Med (Lausanne). 5:138, 2018
                                                6. Magro-Checa C et al: Management of Neuropsychiatric Systemic Lupus Erythematosus: Current Approaches and Future Perspectives. Drugs. 76(4):459-83, 2016
                                                7. Nardone R et al: Longitudinally extensive transverse myelitis in systemic lupus erythematosus: Case report and review of the literature. Clin Neurol Neurosurg. 129C:57-61, 2015
                                                8. Piga M et al: Twenty-year brain magnetic resonance imaging follow-up study in Systemic Lupus Erythematosus: Factors associated with accrual of damage and central nervous system involvement. Autoimmun Rev. ePub, 2015
                                                9. Saison J et al: Systemic lupus erythematosus-associated acute transverse myelitis: manifestations, treatments, outcomes, and prognostic factors in 20 patients. Lupus. 24(1):74-81, 2015
                                                10. Sarbu N et al: Advanced and Conventional Magnetic Resonance Imaging in Neuropsychiatric Lupus. F1000Res. 4:162, 2015
                                                11. Jeong HW et al: Brain MRI in neuropsychiatric lupus: associations with the 1999 ACR case definitions. Rheumatol Int. ePub, 2014
                                                12. Kaichi Y et al: Brain MR findings in patients with systemic lupus erythematosus with and without antiphospholipid antibody syndrome. AJNR Am J Neuroradiol. 35(1):100-5, 2014
                                                13. Steup-Beekman GM et al: Neuropsychiatric manifestations in patients with systemic lupus erythematosus: epidemiology and radiology pointing to an immune-mediated cause. Ann Rheum Dis. 72 Suppl 2:ii76-9, 2013
                                                14. Borowoy AM et al: Neuropsychiatric lupus: the prevalence and autoantibody associations depend on the definition: results from the 1000 faces of lupus cohort. Semin Arthritis Rheum. 42(2):179-85, 2012
                                                15. Curiel R et al: PET/CT imaging in systemic lupus erythematosus. Ann N Y Acad Sci. 1228:71-80, 2011
                                                16. Lim KS et al: Periodic lateralized epileptiform discharges in neuropsychiatric lupus: association with cerebritis in magnetic resonance imaging and resolution after intravenous immunoglobulin. Lupus. 19(6):748-52, 2010
                                                17. Baizabal-Carvallo JF et al: Posterior reversible encephalopathy syndrome as a complication of acute lupus activity. Clin Neurol Neurosurg. 111(4):359-63, 2009
                                                18. Appenzeller S et al: Quantitative magnetic resonance imaging analyses and clinical significance of hyperintense white matter lesions in systemic lupus erythematosus patients. Ann Neurol. 64(6):635-43, 2008
                                                19. Petri M et al: Brain magnetic resonance imaging in newly diagnosed systemic lupus erythematosus. J Rheumatol. 35(12):2348-54, 2008
                                                20. Janardhan V et al: Anticardiolipin antibodies and risk of ischemic stroke and transient ischemic attack: the Framingham cohort and offspring study. Stroke. 35(3):736-41, 2004
                                                21. Jennings JE et al: Value of MRI of the brain in patients with systemic lupus erythematosus and neurologic disturbance. Neuroradiology. 46(1):15-21, 2004
                                                22. Susac JO et al: MRI findings in Susac's syndrome. Neurology. 61(12):1783-7, 2003
                                                Related Anatomy
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                                                Related Differential Diagnoses
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                                                References
                                                Tables

                                                Tables

                                                KEY FACTS

                                                • Terminology

                                                  • Imaging

                                                    • Top Differential Diagnoses

                                                      • Pathology

                                                        • Clinical Issues

                                                          • Diagnostic Checklist

                                                            TERMINOLOGY

                                                            • Abbreviations

                                                              • Systemic lupus erythematosus (SLE)
                                                              • Neuropsychiatric SLE (NPSLE)

                                                            • Definitions

                                                              • Primary NPSLE: If resulting directly from immune-mediated injury
                                                              • Secondary NPSLE: If related to treatment, infections, metabolic disturbances, or other systemic manifestations not related to SLE; ~ 60%

                                                            IMAGING

                                                            • General Features

                                                              • CT Findings

                                                                • MR Findings

                                                                  • Angiographic Findings

                                                                    • Nuclear Medicine Findings

                                                                      • Imaging Recommendations

                                                                        DIFFERENTIAL DIAGNOSIS

                                                                          PATHOLOGY

                                                                          • General Features

                                                                            • Staging, Grading, & Classification

                                                                              • Gross Pathologic & Surgical Features

                                                                                • Microscopic Features

                                                                                  CLINICAL ISSUES

                                                                                  • Presentation

                                                                                    • Demographics

                                                                                      • Natural History & Prognosis

                                                                                        • Treatment

                                                                                          DIAGNOSTIC CHECKLIST

                                                                                          • Consider

                                                                                            • Image Interpretation Pearls

                                                                                              Selected References

                                                                                              1. John TJ et al: SLE pachymeningitis and multiple cranial nerve palsies: a case report and review of the literature. Lupus. 28(9):1154-7, 2019
                                                                                              2. Mvambo N et al: Clinical, neuroimaging and immunological phenotype of South African neuropsychiatric systemic lupus erythematosus patients. Lupus. 28(5):685-94, 2019
                                                                                              3. Yeoh H et al: Relationship between cerebral microbleeds and white matter MR hyperintensities in systemic lupus erythematosus: a retrospective observational study. Neuroradiology. 61(3):265-74, 2019
                                                                                              4. McGlasson S et al: Neurological Disease in Lupus: Toward a Personalized Medicine Approach. Front Immunol. 9:1146, 2018
                                                                                              5. Vivaldo JF et al: Definition of NPSLE: Does the ACR Nomenclature Still Hold? Front Med (Lausanne). 5:138, 2018
                                                                                              6. Magro-Checa C et al: Management of Neuropsychiatric Systemic Lupus Erythematosus: Current Approaches and Future Perspectives. Drugs. 76(4):459-83, 2016
                                                                                              7. Nardone R et al: Longitudinally extensive transverse myelitis in systemic lupus erythematosus: Case report and review of the literature. Clin Neurol Neurosurg. 129C:57-61, 2015
                                                                                              8. Piga M et al: Twenty-year brain magnetic resonance imaging follow-up study in Systemic Lupus Erythematosus: Factors associated with accrual of damage and central nervous system involvement. Autoimmun Rev. ePub, 2015
                                                                                              9. Saison J et al: Systemic lupus erythematosus-associated acute transverse myelitis: manifestations, treatments, outcomes, and prognostic factors in 20 patients. Lupus. 24(1):74-81, 2015
                                                                                              10. Sarbu N et al: Advanced and Conventional Magnetic Resonance Imaging in Neuropsychiatric Lupus. F1000Res. 4:162, 2015
                                                                                              11. Jeong HW et al: Brain MRI in neuropsychiatric lupus: associations with the 1999 ACR case definitions. Rheumatol Int. ePub, 2014
                                                                                              12. Kaichi Y et al: Brain MR findings in patients with systemic lupus erythematosus with and without antiphospholipid antibody syndrome. AJNR Am J Neuroradiol. 35(1):100-5, 2014
                                                                                              13. Steup-Beekman GM et al: Neuropsychiatric manifestations in patients with systemic lupus erythematosus: epidemiology and radiology pointing to an immune-mediated cause. Ann Rheum Dis. 72 Suppl 2:ii76-9, 2013
                                                                                              14. Borowoy AM et al: Neuropsychiatric lupus: the prevalence and autoantibody associations depend on the definition: results from the 1000 faces of lupus cohort. Semin Arthritis Rheum. 42(2):179-85, 2012
                                                                                              15. Curiel R et al: PET/CT imaging in systemic lupus erythematosus. Ann N Y Acad Sci. 1228:71-80, 2011
                                                                                              16. Lim KS et al: Periodic lateralized epileptiform discharges in neuropsychiatric lupus: association with cerebritis in magnetic resonance imaging and resolution after intravenous immunoglobulin. Lupus. 19(6):748-52, 2010
                                                                                              17. Baizabal-Carvallo JF et al: Posterior reversible encephalopathy syndrome as a complication of acute lupus activity. Clin Neurol Neurosurg. 111(4):359-63, 2009
                                                                                              18. Appenzeller S et al: Quantitative magnetic resonance imaging analyses and clinical significance of hyperintense white matter lesions in systemic lupus erythematosus patients. Ann Neurol. 64(6):635-43, 2008
                                                                                              19. Petri M et al: Brain magnetic resonance imaging in newly diagnosed systemic lupus erythematosus. J Rheumatol. 35(12):2348-54, 2008
                                                                                              20. Janardhan V et al: Anticardiolipin antibodies and risk of ischemic stroke and transient ischemic attack: the Framingham cohort and offspring study. Stroke. 35(3):736-41, 2004
                                                                                              21. Jennings JE et al: Value of MRI of the brain in patients with systemic lupus erythematosus and neurologic disturbance. Neuroradiology. 46(1):15-21, 2004
                                                                                              22. Susac JO et al: MRI findings in Susac's syndrome. Neurology. 61(12):1783-7, 2003