Systemic lupus erythematosus: immunopathogenesis of neurologic dysfunction

Moore, Patricia M.; Lisak, Robert P.

doi:10.1007/bf00194099

Cited by 23 publications

(4 citation statements)

References 110 publications

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“…Indeed, it is known that in SLE, the main disease associated to secondary APS, there are multiple causes for seizures, such as nervous central system activity, vasculitis, stroke, and meningoencephalitis [9, 30]. Moreover, all selected patients were negative for lupus-specific autoantibodies.…”

Section: Discussionmentioning

confidence: 99%

Seizures in Primary Antiphospholipid Syndrome: The Relevance of Smoking to Stroke

Carvalho

Pasoto

Appenzeller

2012

Clinical and Developmental Immunology

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Objectives. To evaluate the frequency of seizures in primary antiphospholipid syndrome (PAPS) and their possible clinical and laboratory associations. Methods. Eighty-eight PAPS patients (Sydney's criteria) were analyzed by a standard interview, physical examination and review of medical charts. Risk factors for seizures, clinical manifestations, associated comorbidities, and antiphospholipid antibodies were evaluated. Results. Nine (10.2%) patients with seizures were identified, 77.8% had convulsions onset after PAPS diagnosis. Mean age, gender, and race were comparable in groups with or without seizures. Interestingly, a higher frequency of current smoking (44.4 versus 10.1%, P = 0.019) was observed in the first group. Stroke, Sneddon's syndrome, and livedo reticularis were more frequent in PAPS patients with seizures than those without seizures, although not statistically significant (P > 0.05). Comparison between patients with seizures onset after PAPS diagnosis (n = 7) and those without convulsions (n = 79) demonstrated a higher frequency of current smoking (42.9 versus 10%, P = 0.042) and stroke in the first group (71.4 versus 30.4%, P = 0.041). Regression analysis confirmed that smoking (P = 0.030) and stroke (P = 0.042) were independently associated to seizures. Conclusion. About 10.2% of PAPS patients had convulsions, predominantly after PAPS diagnosis, and seizures were associated to current smoking and stroke.

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Section: Discussionmentioning

confidence: 99%

Seizures in Primary Antiphospholipid Syndrome: The Relevance of Smoking to Stroke

Carvalho

Pasoto

Appenzeller

2012

Clinical and Developmental Immunology

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“…It has been reported that immune complexes, autoantibodies, and cytokines cause neurological disorders in SLE with direct effects and that vasculopathy developing in arterioles that feed the basal ganglia, coagulopathy, and bleeding disorders cause neurological disorders with indirect effects (2,5).…”

Section: Discussionmentioning

confidence: 99%

A Rare Presentation of Systemic Lupus Erythematosus during Childhood: Chorea

Demir¹,

Küçükkoç²,

Göknar³

et al. 2017

Bezmialem Science

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Systemic lupus erythematosus (SLE) is a systemic, chronic, inflammatory, autoimmune disease characterized by multi-organ and -system involvement such as the kidneys and hematologic and central nervous systems. The clinical presentation of the disease is extremely variable. Systemic findings, nephritis, neuropsychiatric disease, and cytopenia are more common in children than in adults at presentation. Chorea typically manifests with jerky, involuntary, and purposeless movements, involving the face, four limbs, and neck. In addition, patients suffer from dysarthria, gait disturbance, and neuropsychiatric symptoms. Chorea can be the first finding of SLE during childhood. Herein we reported a 13-year female patient who presented with movement and speech disorders and who was diagnosed chorea-related SLE.

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“…Systemic lupus erythematosus (SLE) is a chronic inflammatory autoimmune disease that affects every organ and system in the body. The loss of tolerance in the immune system results in autoantibody production, immune complex deposition, and complement activation, which lead to systemic inflammation and target tissue damage ( 1 ). Genetic factors are essential in SLE susceptibility, according to family studies and the concordance rate between twins ( 2 ).…”

Section: Introductionmentioning

confidence: 99%

The Chromatin Accessibility Landscape of Peripheral Blood Mononuclear Cells in Patients With Systemic Lupus Erythematosus at Single-Cell Resolution

Hong

et al. 2021

Front. Immunol.

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ObjectiveSystemic lupus erythematosus (SLE) is a complex autoimmune disease, and various immune cells are involved in the initiation, progression, and regulation of SLE. Our goal was to reveal the chromatin accessibility landscape of peripheral blood mononuclear cells (PBMCs) in SLE patients at single-cell resolution and identify the transcription factors (TFs) that may drive abnormal immune responses.MethodsThe assay for transposase accessible chromatin in single-cell sequencing (scATAC-seq) method was applied to map the landscape of active regulatory DNA in immune cells from SLE patients at single-cell resolution, followed by clustering, peak annotation and motif analysis of PBMCs in SLE.ResultsPeripheral blood mononuclear cells were robustly clustered based on their types without using antibodies. We identified twenty patterns of TF activation that drive abnormal immune responses in SLE patients. Then, we observed ten genes that were highly associated with SLE pathogenesis by altering T cell activity. Finally, we found 12 key TFs regulating the above six genes (CD83, ELF4, ITPKB, RAB27A, RUNX3, and ZMIZ1) that may be related to SLE disease pathogenesis and were significantly enriched in SLE patients (p <0.05, FC >2). With qPCR experiments on CD83, ELF4, RUNX3, and ZMIZ1 in B cells, we observed a significant difference in the expression of genes (ELF4, RUNX3, and ZMIZ1), which were regulated by seven TFs (EWSR1-FLI1, MAF, MAFA, NFIB, NR2C2 (var. 2), TBX4, and TBX5). Meanwhile, the seven TFs showed highly accessible binding sites in SLE patients.ConclusionsThese results confirm the importance of using single-cell sequencing to uncover the real features of immune cells in SLE patients, reveal key TFs in SLE-PBMCs, and provide foundational insights relevant for epigenetic therapy.

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Systemic lupus erythematosus: immunopathogenesis of neurologic dysfunction

Cited by 23 publications

References 110 publications

Seizures in Primary Antiphospholipid Syndrome: The Relevance of Smoking to Stroke

Seizures in Primary Antiphospholipid Syndrome: The Relevance of Smoking to Stroke

A Rare Presentation of Systemic Lupus Erythematosus during Childhood: Chorea

The Chromatin Accessibility Landscape of Peripheral Blood Mononuclear Cells in Patients With Systemic Lupus Erythematosus at Single-Cell Resolution

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