Neuropsychiatric Manifestations of Antiphospholipid Syndrome—A Narrative Review

Man, Yik Long; Sanna, Giovanni

doi:10.3390/brainsci12010091

Cited by 17 publications

(11 citation statements)

References 153 publications

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“…The estimated incidence and prevalence of APS ranged between 1 and 2 cases per 100,000 and 40 and 50 cases per 100,000 respectively (5). Patients may also develop kidney disease, digestive tract ischemia, pulmonary manifestations, and cardiac and neuropsychiatric symptoms (6,7). The most common complication of obstetric APS (OAPS) is recurrent miscarriage in the first trimester, which has been attributed to inhibition of the proliferation of trophoblastic cells (8).…”

Section: Introductionmentioning

confidence: 99%

Preliminary Study on the Imbalance Between Th17 and Regulatory T Cells in Antiphospholipid Syndrome

Yan

et al. 2022

Front. Immunol.

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ObjectivePatients with antiphospholipid syndrome (APS) have immune cell abnormalities that remain poorly understood. This study compared primary APS (PAPS) and secondary APS (SAPS) patients with healthy controls with respect to peripheral blood lymphocytes, CD4+T cell subsets, and cytokine levels. The correlation between antiphospholipid antibody titres and T helper 17 (Th17) and T regulatory (Treg) cell subsets was also analyzed, together with the correlations between cytokine profiles and the clinical characteristics of APS patients.MethodsThe retrospective study population consisted of 67 APS patients (12 with PAPS, 55 with SAPS) and 40 healthy controls. Absolute numbers of peripheral blood lymphocyte subsets and CD4+ T cell subsets were detected by flow cytometry, and serum cytokine levels by flow cytometry bead array.ResultsPatients with SAPS had lower absolute values of T, B and CD4+T cells than the healthy control group, while only natural killer (NK) cell levels were decreased in patients with PAPS. Absolute numbers of T, B, NK, and CD4+T cells were significantly higher in the PAPS than SAPS group. The trends in CD4+T cell subsets were the same in PAPS and SAPS patients as in healthy controls, with increased Th1, decreased Th2, and decreased Treg levels, and thus an increased Th17/Treg ratio. Th2, Th17, and Treg cell counts were higher in the PAPS than SAPS group. Cytokine analysis showed that only IL-10 levels differed between the two APS groups. However, the levels of all of the studied cytokines were higher in APS patients than healthy controls, and correlated with the clinical characteristics of the patients. In the PAPS group, the titres of two autoantibodies correlated positively with the Th17/Treg ratio and negatively with the levels of D-dimer and Treg subsets.ConclusionsOur study clearly showed that APS patients have immune disturbances, the most prominent of which is an increase in the Th17/Treg ratio, due to a decrease in the number of Treg cells. These abnormalities may be involved in the occurrence and progression of APS. An additional finding was a higher level of peripheral blood lymphocytes in PAPS than SAPS patients, which may be related to the immunosuppressive treatment of SAPS patients.

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Section: Introductionmentioning

confidence: 99%

Preliminary Study on the Imbalance Between Th17 and Regulatory T Cells in Antiphospholipid Syndrome

Yan

et al. 2022

Front. Immunol.

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“…Another retrospective study that examined data from 1980 to 2009 in Minnesota found that rheumatoid arthritis patients were 1.37 times more likely to develop a dementia disorder than those without the disease [ 8 ]. Similarly, about 2.5% of patients diagnosed with APS develop a dementia disorder [ 5 ]. In recent years, further information has emerged supporting a physiological link between chronic inflammation states, such as rheumatoid arthritis and APS, and the development of dementia disorders, including frontotemporal dementia [ 9 ].…”

Section: Discussionmentioning

confidence: 99%

“…In doing so, the disorder’s effect is systemic, involving an array of manifestations ranging from cardiac to renal and to neuropsychiatric manifestations. Among the neuropsychiatric manifestations are well-characterized complications of cerebral ischemia and less understood non-thrombotic manifestations of cognitive dysfunction and dementia [ 5 ]. Conventionally, treatment options for clinical manifestations that are a consequence of thrombosis are warfarin anticoagulation and for non-thrombotic symptoms are antiplatelet therapy.…”

Section: Introductionmentioning

confidence: 99%

The Link Between Frontotemporal Dementia and Autoimmunity: A Case Presentation and Literature Review

Corzo¹,

Farabi²,

Lahoti³

2022

Cureus

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Dementia disorders are an important public health issue and thus of particular clinical importance. Frontotemporal dementia, although less prevalent than Alzheimer’s disease, presents in a significant number of cases in younger populations. Yet, it is a comparatively rare disease process, with a low yearly incidence. Frontotemporal dementia remains an exciting and ever-evolving area of research with most recent studies investigating the role of inflammation in the degeneration pathognomonic of the disease. Here, we describe a case that highlights the connection between inflammation and neurodegeneration. Specifically, we examine a patient with long-standing rheumatoid arthritis and antiphospholipid syndrome who developed frontotemporal dementia, potentially as a result of the chronic inflammatory state.

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“…aPLAs consist of the anti-cardiolipin (aCL) and anti-beta-2-glycoprotein I antibodies (aβ2GPI), and the lupus anticoagulant (LAC), which may cause disease by processes that culminate in thromboembolic phenomena, which may underpin their role in NPSLE. They may be found in 30–50% of SLE patients, up to half of whom may go on to develop features of the antiphospholipid antibody syndrome, and may also present with both focal or diffuse NPSLE manifestations ( 30 , 31 ). Cognitive disorders have been reported in 54% of aPL-positive SLE patients, compared to 7% of those seronegative ( 32 ), and has shown associations with aCL or LAC ( 33 ).…”

Section: Shortcomings Of the Current Diagnostic Algorithmmentioning

confidence: 99%

The conundrum of neuropsychiatric systemic lupus erythematosus: Current and novel approaches to diagnosis

et al. 2023

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Recognising neuropsychiatric involvement by systemic lupus erythematosus (SLE) is of growing importance, however many barriers to this exist at multiple levels of our currently available diagnostic algorithms that may ultimately delay its diagnosis and subsequent treatment. The heterogeneous and non-specific clinical syndromes, serological and cerebrospinal fluid (CSF) markers and neuroimaging findings that often do not mirror disease activity, highlight important research gaps in the diagnosis of neuropsychiatric SLE (NPSLE). Formal neuropsychological assessments or the more accessible screening metrics may also help improve objective recognition of cognitive or mood disorders. Novel serum and CSF markers, including autoantibodies, cytokines and chemokines have also shown increasing utility as part of diagnosis and monitoring, as well as in distinguishing NPSLE from SLE patients without SLE-related neuropsychiatric manifestations. Novel neuroimaging studies also expand upon our existing strategy by quantifying parameters that indicate microarchitectural integrity or provide an assessment of neuronal function. Some of these novel markers have shown associations with specific neuropsychiatric syndromes, suggesting that future research move away from considering NPSLE as a single entity but rather into its individually recognized neuropsychiatric manifestations. Nevertheless, it is likely that a composite panel of these investigations will be needed to better address the gaps impeding recognition of neuropsychiatric involvement by SLE.

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Neuropsychiatric Manifestations of Antiphospholipid Syndrome—A Narrative Review

Cited by 17 publications

References 153 publications

Preliminary Study on the Imbalance Between Th17 and Regulatory T Cells in Antiphospholipid Syndrome

Preliminary Study on the Imbalance Between Th17 and Regulatory T Cells in Antiphospholipid Syndrome

The Link Between Frontotemporal Dementia and Autoimmunity: A Case Presentation and Literature Review

The conundrum of neuropsychiatric systemic lupus erythematosus: Current and novel approaches to diagnosis

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