Abstract 13131: Cardiovascular Manifestations and Outcomes in Systemic Lupus Erythematosus: The Cleveland Clinic Contemporary Experience

Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Tom Kai Ming Wang ◽  
Nicholas Chan ◽  
Mohamed Khayata ◽  
Patrick Flanagan ◽  
Richard A Grimm ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Cleveland Clinic ◽  
Cardiac Events ◽  
Systemic Lupus ◽  
Pericardial Disease ◽  
Tertiary Referral Center ◽  
Multiple Organs ◽  
Ischemic Attack ◽  
Cardiac Manifestations

Background: Systemic lupus erythematosus (SLE) is an autoimmune connective tissue disease affecting multiple organs including the heart, which when affected portends poor prognosis. Given the advances in therapies and vigilance for cardiovascular screening for SLE patients, we evaluated the contemporary cardiovascular characteristics, manifestations and outcomes in SLE patients at our tertiary referral center. Methods: Consecutive patients from the prospective SLE bio-repository at our center between October 2012 and March 2020 were included. Cardiovascular data pertaining to manifestations, investigations, management and outcomes were collected. Results: 258 SLE patients were studied, with mean age 42.2 ± 14.7 years and 233 (90.3%) females. Cardio-respiratory symptoms were present in 92 (35.7%), most commonly dyspnea in 62 (24.0%) and chest pain in 53 (20.5%). Cardiac manifestations occurred in 97 (37.6%) patients, with pericardial disease in 38 (14.7%), valvular disease in 20 (7.8%), stroke/transient ischemic attack in 20 (7.8%), coronary heart disease in 17 (6.6%) and heart failure hospitalizations in 13 (5.0%) (Table 1). During a mean follow-up of 3.0 ± 2.2 years, there were 5 (1.9%) deaths, 19 (7.4%) developed cardiac events (table 1), 6 (2.3%) had cardiovascular procedures (3 cardiac surgeries, 2 percutaneous interventions and 1 device), and 44 (17.1%) had SLE-related hospitalizations. Conclusion: Cardiac manifestations remain prevalent in SLE, especially for pericardial, valvular and atherosclerotic diseases. With appropriate contemporary SLE and cardiovascular management, subsequent medium term adverse cardiovascular outcomes are low.

scholarly journals Use of echocardiography at diagnosis and detection of acute cardiac disease in youth with systemic lupus erythematosus

Lupus ◽  
2018 ◽  
Vol 27 (8) ◽  
pp. 1348-1357 ◽  
Author(s):  
J C Chang ◽  
A M Knight ◽  
R Xiao ◽  
L M Mercer-Rosa ◽  
P F Weiss
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Cardiac Disease ◽  
Baseline Period ◽  
Administrative Database ◽  
Systemic Lupus ◽  
Pericardial Disease ◽  
Cardiac Manifestations ◽  
Valvular Insufficiency ◽  
New Onset

Objectives There are no guidelines on the use of echocardiography to detect cardiac manifestations of childhood-onset systemic lupus erythematosus (SLE). We quantify the prevalence of acute cardiac disease in youth with SLE, describe echocardiogram utilization at SLE diagnosis, and compare regional echocardiogram use with incident cardiac diagnoses. Methods Using the Clinformatics® DataMart (OptumInsight, Eden Prairie, MN) de-identified United States administrative database from 2000 to 2013, we identified youth ages 5–24 years with new-onset SLE (≥3 ICD-9 SLE codes 710.0, > 30 days apart) and determined the prevalence of diagnostic codes for pericardial disease, myocarditis, endocarditis, and valvular insufficiency. Multiple logistic regression was used to identify factors associated with echocardiography during the baseline period, up to one year before or six months after SLE diagnosis. We calculated a regional echocardiogram utilization index, which is the ratio of observed use over the mean predicted probability based on all available baseline characteristics. Spearman’s rank correlation coefficient was used to evaluate the association between regional echocardiogram utilization indices and percentage of imaged youth diagnosed with their first cardiac manifestation following echocardiography. Results Among 699 youth with new-onset SLE, 18% had ≥ 1 diagnosis code for acute cardiac disease, of which valvular insufficiency and pericarditis were most common. Twenty-five percent of all youth underwent echocardiogram during the baseline period. Regional echocardiogram use was positively correlated with the percentage of imaged youth found to have cardiac disease (ρ = 0.71, p = 0.05). There was up to a five-fold difference in adjusted odds of baseline echocardiography between low- and high-utilizing regions (OR = 0.19, p = 0.007). Conclusion Nearly one-fifth of youth with new-onset SLE have acute cardiac manifestations; however, use of echocardiograms at SLE diagnosis is highly variable. There may be incremental diagnostic value to early use of echocardiography, but prospective studies are needed to determine whether greater use of echocardiograms modifies outcomes.

scholarly journals Epigenetic Contribution and Genomic Imprinting Dlk1-Dio3 miRNAs in Systemic Lupus Erythematosus

Genes ◽  
2021 ◽  
Vol 12 (5) ◽  
pp. 680
Author(s):  
Rujuan Dai ◽  
Zhuang Wang ◽  
S. Ansar Ahmed
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Dna Methylation ◽  
Lupus Erythematosus ◽  
Critical Role ◽  
Methylation Status ◽  
Transcriptional Level ◽  
Dna Hypomethylation ◽  
Systemic Lupus ◽  
Multiple Organs ◽  
Genetic Imprinting

Systemic lupus erythematosus (SLE) is a multifactorial autoimmune disease that afflicts multiple organs, especially kidneys and joints. In addition to genetic predisposition, it is now evident that DNA methylation and microRNAs (miRNAs), the two major epigenetic modifications, are critically involved in the pathogenesis of SLE. DNA methylation regulates promoter accessibility and gene expression at the transcriptional level by adding a methyl group to 5′ cytosine within a CpG dinucleotide. Extensive evidence now supports the importance of DNA hypomethylation in SLE etiology. miRNAs are small, non-protein coding RNAs that play a critical role in the regulation of genome expression. Various studies have identified the signature lupus-related miRNAs and their functional contribution to lupus incidence and progression. In this review, the mutual interaction between DNA methylation and miRNAs regulation in SLE is discussed. Some lupus-associated miRNAs regulate DNA methylation status by targeting the DNA methylation enzymes or methylation pathway-related proteins. On the other hand, DNA hyper- and hypo-methylation are linked with dysregulated miRNAs expression in lupus. Further, we specifically discuss the genetic imprinting Dlk1-Dio3 miRNAs that are subjected to DNA methylation regulation and are dysregulated in several autoimmune diseases, including SLE.

scholarly journals POS0709 LUPUS FOG IS NOT DISSOCIATIVE FOG

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 604.1-604
Author(s):  
R. Monahan ◽  
A. Blonk ◽  
H. Middelkoop ◽  
M. Kloppenburg ◽  
T. Huizinga ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Neuropsychiatric Symptoms ◽  
Adult Population ◽  
Systemic Lupus ◽  
Tertiary Referral Center ◽  
Neuropsychiatric Systemic Lupus Erythematosus ◽  
Dissociative Symptoms ◽  
Dissociative Experience ◽  
Dissociative Experience Scale

Background:The presence of a ‘fog’ is frequently reported by patients with systemic lupus erythematosus (SLE). However, little is known about this lupus fog: it is thought to be a result of cognitive dysfunction, but fogs can also be the result of dissociation. The Dissociative Experience Scale-II (DES) is a standardized tool to study dissociation. In the general adult population, scores range from 4.4-14.1-3Objectives:We aimed to study the prevalence of dissociative symptoms including dissociative fog in patients with SLE and neuropsychiatric symptoms.Methods:Patients visiting the tertiary referral center for neuropsychiatric systemic lupus erythematosus (NPSLE) of the LUMC between 2007-2019 were included. All patients underwent a standardized multidisciplinary assessment. Patients were classified as NPSLE if neuropsychiatric symptoms were attributed to SLE and immunosuppressive or anticoagulant therapy was initiated, otherwise patients were classified as minor/non-NPSLE. Dissociation was studied using the DES. The DES separates different types of dissociative symptoms: amnesia, absorption/imagination and derealization/depersonalization. It also contains one question regarding evaluating the presence of a dissociative fog: “Some people sometimes feel as if they are looking at the world through a fog, so that people and objects appear far away or unclear”. All statements (n = 28) regarding dissociative symptoms are rated from ‘none of the time’ to ‘all of the time’ (0-100%); scores >25 are considered abnormal. A multiple regression analysis (MRA) were performed to compare dissociation in patients with and without NPSLE. DES results are presented as median (range) and MRA as odds ratio (OR) and 95% confidence interval (CI).Results:DES questionnaires were available for 337 patients, of which 97 had the diagnosis NPSLE (29%). Mean age in patients with NPSLE was 41 ± 13 years and 84% was female. In minor/non-NPSLE, median age was 44 ± 14 years and 87% was female.Median dissociation was 7 (0-75) and did not differ between patients with minor/non-NPSLE and NPSLE (OR: 1.0 (95% CI: -0.9; 1.1)). The most common type of dissociation was absorption/imagination (median: 12, range 0-75) and depersonalization/derealization was infrequent (median: 1, range 0-84). 43 patients (13%) had an abnormal score (>25) on the dissociative fog question.Conclusion:Dissociative symptoms are within normal range in patients with SLE and neuropsychiatric symptoms, regardless of underlying etiology. Dissociative fog seems uncommon and therefore lupus fog is most likely not the result of dissociation.References:[1]Bernstein EM and Putnam FW. Development, reliability, and validity of a dissociation scale. J Nerv Ment Dis 1986; 174: 727-735. 1986/12/01. DOI: 10.1097/00005053-198612000-00004.[2]Maaranen P, Tanskanen A, Honkalampi K, et al. Factors associated with pathological dissociation in the general population. Aust N Z J Psychiatry 2005; 39: 387-394. 2005/04/30. DOI: 10.1080/j.1440-1614.2005.01586.x.[3]van IJzendoorn MH and Schuengel C. The measurement of dissociation in normal and clinical populations: Meta-analytic validation of the Dissociative Experiences Scale (DES). Clinical Psychology Review 1996; 16: 365-382. DOI: 10.1016/0272-7358(96)00006-2.Table 1.Presence of dissociation in patients with SLE and neuropsychiatric symptomsTotal cohort(n = 337)Minor/non-NPSLE(n = 240)NPSLE(n = 97)DES (median, range)Total questionnaire7 (0 - 75)8 (0 - 66)6 (0 – 75)Amnesia5 (0 - 76)5 (0 - 68)4 (0 - 76)Absorption/imagination12 (0 – 75)13 (0 – 75)10 (0 – 73)Depersonalization/derealization1 (0 – 84)1 (0 – 73)1 (0 – 84)Dissociative fog* 0 (0-100)0 (0-100)0 (0-100)DES = Dissociative Experience Scale NPSLE = neuropsychiatric systemic lupus erythematosus.*Dissociative fog is question 28 of the DES-IIDisclosure of Interests:None declared

Utilization of glucocorticoids among White and Black patients with systemic lupus erythematosus: Observations from the enrollment visit of a prospective registry

Lupus ◽  
2021 ◽  
pp. 096120332110558
Author(s):  
James K Sullivan ◽  
Emily A Littlejohn
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Logistic Regression ◽  
Health Outcomes ◽  
Lupus Erythematosus ◽  
Cleveland Clinic ◽  
Systemic Lupus ◽  
Black And White ◽  
Black Patients ◽  
White Patients

Background Black patients with systemic lupus erythematosus (SLE) face higher rates of morbidity and mortality compared to White patients. Long-term glucocorticoid use has been associated with worse health outcomes among patients with SLE. We sought to quantify chronic glucocorticoid use among Black and White patients with SLE within a prospective registry. Methods Using enrollment data from a registry at a large academic institution, we compared glucocorticoid use among Black and White patients with SLE. Multivariable logistic regression of race and glucocorticoid use was performed, adjusting for covariates exhibiting a bivariate association with glucocorticoids at significance level p < 0.10. Results 114 White participants (mean age 45; standard deviation (SD) 15) and 59 Black participants (mean age 42; SD 14) were analyzed. White participants had mean SLEDAI-2K score of 3.7 (SD 5.2). Black participants had mean SLEDAI-2K scores of 6.3 (SD 6.0). Among Black participants, 43 (72%) utilized glucocorticoids compared to White participants 39 (34%) (unadjusted odds ratio (OR) 5.17; 95% confidence interval (CI) 2.59–10.33). We did not observe differences between unadjusted hydroxychloroquine (OR 0.69; 95% CI 0.28–1.65) or conventional disease-modifying anti-rheumatic drug (cDMARD) (OR 1.07; 95% CI 0.57–2.01) utilization among Black and White participants. SLEDAI-2K, disability, recent hospitalization, and past or present hydroxychloroquine or cDMARD use were included in a logistic regression model. Adjusting for covariates, Black participants were more likely to be on glucocorticoids (adjusted OR 5.69; 95% CI 2.17–14.96); p = 0.0004). Conclusion Adjusting for disease activity and other medications, Black patients had more exposure to chronic glucocorticoids than White patients in the Cleveland Clinic SLE registry. These patients may face increased glucocorticoid-related morbidity, which could contribute significantly to long-term health outcomes and utilization of health care resources. Future research in larger, more diverse registries should be conducted to further characterize patterns of glucocorticoid use.

scholarly journals The Role ofγδT Cells in Systemic Lupus Erythematosus

2016 ◽  
Vol 2016 ◽  
pp. 1-8 ◽  
Author(s):  
Meng Wu ◽  
Jinhua Yang ◽  
Xiaofeng Li ◽  
Junwei Chen
Keyword(s):  
Systemic Lupus Erythematosus ◽  
T Cells ◽  
Autoimmune Disease ◽  
Lupus Erythematosus ◽  
Systemic Lupus ◽  
Multiple Organs ◽  
Minor Population ◽  
Neuronal Tissues ◽  
A Minor

Systemic lupus erythematosus (SLE) is an autoimmune disease that is characterized by the overproduction of autoantibodies against an array of nuclear and cytoplasmic antigens and affects multiple organs, such as the skin, joints, kidneys, and neuronal tissues. T cells have been recognized as important players in the development of SLE due to their functions in cytokine secretion, antigen presentation, and supporting B cells for antibody production.γδT cells are a minor population of T cells that play important roles in infection and tumor-associated disease. In recent years, the role ofγδT cells in autoimmune diseases has been investigated. In this review, we discussed the role ofγδT cells in the pathogenesis of SLE.

Association of IgA Anti-ß2 Glycoprotein I with Clinical and Laboratory Manifestations of Systemic Lupus Erythematosus

2010 ◽  
Vol 38 (1) ◽  
pp. 64-68 ◽  
Author(s):  
TARANEH MEHRANI ◽  
MICHELLE PETRI
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Venous Thrombosis ◽  
Deep Venous Thrombosis ◽  
Lupus Erythematosus ◽  
Classification Criteria ◽  
Systemic Lupus ◽  
Superficial Thrombophlebitis ◽  
Glycoprotein I ◽  
High Prevalence ◽  
Ischemic Attack

Objective.IgA isotypes of anticardiolipin and anti-ß2 glycoprotein I (anti-ß2-GPI) are omitted from the revised antiphospholipid syndrome (APS) classification criteria. Multiple studies have found a high prevalence of IgA anti-ß2-GPI in systemic lupus erythematosus (SLE). We determined the frequency and associations of IgA anti-ß2-GPI in a cohort of patients with SLE.Methods.Anti-ß2-GPI was measured in 796 patients with SLE (93% women, 53% white, 38% African American, mean age 45 yrs). IgA anti-ß2-GPI (> 20 phospholipid units) was found in 20%. Using a cohort database, associations with cumulative thrombotic and other manifestations were determined.Results.Of patients with SLE who demonstrated IgA anti-ß2-GPI positivity, about 6% had transient ischemic attack (p = 0.070), 4% had superficial thrombophlebitis (p = 0.647), 20% had deep venous thrombosis (p = 0.003), 4% had other venous thrombosis (p = 0.827), 12% had stroke (p = 0.050), and 1% had myocardial infarction (p = 0.397).Conclusion.IgG anti-ß2-GPI has the strongest association with thrombosis in SLE. However, IgA anti-ß2-GPI was more strongly associated with deep venous thrombosis and with stroke than was IgM. These results indicate that assessment of IgA anti-ß2-GPI is associated with thrombosis in SLE, and that the classification criteria for APS should be revised to include IgA anti-ß2-GPI in patients with SLE.

scholarly journals Clinical subgroup clustering analysis in a systemic lupus erythematosus cohort from Western Pennsylvania

2020 ◽  
Author(s):  
Patrick Coit ◽  
Lacy Ruffalo ◽  
Amr H Sawalha
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Clustering Analysis ◽  
Medical Center ◽  
Classification Criteria ◽  
Clinical Disease ◽  
Systemic Lupus ◽  
Oral Ulcers ◽  
Multiple Organs ◽  
Western Pennsylvania

AbstractObjectiveSystemic lupus erythematosus (SLE) is a complex heterogenous autoimmune disease that can affect multiple organs. We performed clinical clustering analysis to describe a lupus cohort from the University of Pittsburgh Medical Center.MethodsA total of 724 patients who met the ACR classification criteria for SLE were included in this study. Clustering was performed using the ACR classification criteria and the partitioning around medoid method. Correlation analysis was performed using the Spearman’s Rho test.ResultsPatients with SLE in our cohort identify 3 district clinical disease subsets. Patients in Cluster 1 were significantly more likely to develop renal and hematologic involvement, and had overrepresentation in African-American and male lupus patients. Clusters 2 and 3 identified a milder disease, with a significantly less likelihood of organ complications. Patients in Cluster 2 are characterized by malar rash and photosensitivity, while patients in Cluster 3 are characterized by oral ulcers which is present in ∼90% of patients within this cluster. The presence of photosensitivity or oral ulcers appears to be protective against the development of lupus nephritis in our cohort.ConclusionsWe describe a large cohort of SLE from Western Pennsylvania and identify 3 distinct clinical disease subgroups. Clustering analysis might help to better manage and predict disease complications in heterogenous diseases like lupus.

scholarly journals HLA-DRB1*04 as a Risk Allele to Systemic Lupus Erythematosus and Lupus Nephritis in the Malay Population of Malaysia

2021 ◽  
Vol 7 ◽  
Author(s):  
Malarvili Selvaraja ◽  
Voon Kin Chin ◽  
Maha Abdullah ◽  
Masita Arip ◽  
Syafinaz Amin-Nordin
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Nephritis ◽  
Lupus Erythematosus ◽  
Activity Index ◽  
Disease Activity Index ◽  
Systemic Lupus ◽  
Mortality And Morbidity ◽  
Curative Therapy ◽  
Multiple Organs ◽  
Increased Risk

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease afflicting multiple organs. Lupus nephritis (LN) is a serious complication of SLE and remains a major cause of mortality and morbidity. Curative therapy remains unavailable as etiology from genetic and environmental factors is still unclear. The present study was conducted to elucidate the link between HLA-DRB1 gene polymorphisms with SLE and LN through clinical and laboratory/biological presentations in a population of Malaysian Malay females with SLE. A total of 100 Malay female SLE patients inclusive of 70 SLE patients without LN and 30 patients with LN were included in this study. HLA-DRB1 allele examination in SLE patients was performed using PCR-SSO, and the alleles' frequencies were compared with 951 publicly available datasets representing Malay healthy controls in Malaysia. Cytokines and free radical levels were detected by ELISA and bead-based multiplexed Luminex assays. The association between HLA-DRB1 alleles with clinical and serological manifestations and immune mediators was analyzed using different statistical approaches whenever applicable. Our study showed that HLA-DRB1*0405, HLA-DRB1*1502, and HLA-DRB1*1602 were associated with the increased risk of SLE while HLA-DRB1*1201 and HLADRB1*1202 alleles were associated with a lower risk of SLE development. Furthermore, HLA-DRB1*04 showed significant association to LN and arthritis while HLA-DRB1*15 was significantly associated with oral ulcer in Malay SLE patients. Association analysis of HLA-DRB1*04 with clinical and biological factors revealed that HLA-DRB1*04 was significantly associated with Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) scores, anti-nuclear antibody (ANA), C-reactive protein (CRP) in the blood, and total protein in the urine. SLE carriers with the HLA-DRB1*04 allele were significantly correlated to the increased levels of cytokines (IFN-y, GM-CSF, IL-17F, IL-18, IL-21, and VEGF) and were significantly showing negative correlation to IL-5 and free radicals (LPO and catalase enzyme) levels compared to SLE carriers without HLA-DRB1*04 allele. The results suggested that disease severity in SLE may be determined by HLA-DRB1 alleles. The risk of HLA-DRB1*04 allele with LN was supported by the demonstration of an intense inflammatory response in Malay SLE patients in Malaysia. More studies inclusive of a larger and multiple SLE cohorts in the future are warranted to validate these findings.

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