Mortality In Systemic Lupus Erythematosus Research Articles

Mortality and causes of death in systemic lupus erythematosus over the last decade: Data from a large population-based study

ObjectiveTo assess mortality rates (MRs), standardized mortality ratios, and causes of death in systemic lupus erythematosus (SLE) in a population-based study. MethodsWe analyzed linked administrative health databases of the Veneto Region (Italy, 4,900,000 residents). SLE was defined by any hospital diagnosis or healthcare copayment exemption for SLE. We analyzed mortality from January 1st, 2012, until December 31st, 2021. MRs per 1000 were stratified by year, sex, and age group. Standardized mortality ratios were derived by comparing MRs of the general regional population. Causes of death were coded using the ICD-10 coding system and they were grouped in: SLE, infectious diseases, cardiovascular diseases (CVD), cancer, or others. ResultsAmong 4283 SLE prevalent cases, 603 deaths occurred, corresponding to an average annual standardized MR of 18.6 per 1000 person/year (95% CI 17.0-20.2). Out of 1092 incident SLE patients, 90 died with a peak in the first year after diagnosis (MR 26.5 per 10,000 person/month). Standardized mortality ratio was 2.65 (95% CI 2.13-3.26) overall, and highest among younger patients (<45 years: 5.59, 95% CI 2.05-12.4). Five- and 8-year survival were 91% and 89%, respectively. About half of the deaths had CVD or cancer as underlying cause, whereas infections were less frequently reported. ConclusionsAlthough the medium-term survival since diagnosis is good, SLE mortality is still higher than that of the general population, especially in youngest patients. Nowadays, CVD seems to be the major cause of deaths in SLE, whereas infections account for a low proportion of deaths, at least in Western countries.

Refractory Systemic Lupus Erythematosus: Identification and Pharmacological Management.

Systemic lupus erythematosus (SLE) is characterized by an aberrant immune response, leading to an extremely heterogeneous clinical presentation, potentially affecting different systems and organs. Despite the fact that SLE mortality has greatly decreased since the introduction of steroids, some forms of refractory/severe SLE still have the potential to result in permanent organ damage as well as increased mortality and morbidity. Furthermore, SLE patients with multiple comorbidities may face a clinical conundrum and have a bad prognosis. An improved prognosis for severe refractory SLE depends on prompt and appropriate treatment. Due to the scarcity of solid data from a well-characterized group of patients with refractory/severe SLE coming from randomized controlled studies, this review aims to shed light on this with real-world evidence from clinical research performed at our Unit, the University Center of Excellence on Nephrologic, Rheumatologic and Rare Diseases with Nephrology and Dialysis Unit and Center of Immuno-Rheumatology and Rare Diseases (CMID) (Turin, Italy). In order to determine the key clinical and prognostic features, and therapeutic approaches for severe and/or refractory SLE, our experience will be described together with existing literature, primarily focused on dermatological, neuropsychiatric, and renal symptoms.

The association of systemic lupus erythematosus with short-term mortality in sepsis: a population-level analysis.

Systemic lupus erythematosus (SLE) is associated with higher risks of sepsis and sepsis-related mortality compared to the general population. However, the prognostic impact of SLE in sepsis is uncertain. We used statewide data to identify hospitalizations aged ≥18 years in Texas with sepsis, with and without SLE during 2014-2017. Multilevel logistic regression with propensity adjustment (primary model), propensity score matching, and multivariable logistic regression without propensity adjustment were used to estimate the association of SLE with short-term mortality (defined as in-hospital mortality or discharge to hospice) among sepsis hospitalizations. Among 283,025 sepsis hospitalizations, 2933 (1.0%) had SLE. Compared to sepsis hospitalizations without SLE, those with SLE were younger (aged ≥65 years, 25.0% vs 57.0%) and had higher burden of comorbidities (mean Deyo comorbidity index 3.0 vs 2.6). Short-term mortality of sepsis hospitalizations with and without SLE was 22.9% vs 31.3%. SLE remained associated with lower short-term mortality on the secondary models, but not on the primary one (adjusted odds ratio: 0.905; 95% confidence interval: 0.817-1.001). When in-hospital mortality was used as secondary outcome, SLE was associated with mortality only on propensity score matching. The increased sepsis-related mortality in SLE is driven by higher risk of sepsis, but not by higher case fatality among septic patients. SLE may be associated with lower risk of mortality among septic patients, but further studies are needed due to heterogeneity of the prognostic associations across models.

Seronegative Full-house Nephropathy with Crohn's Disease.

Systemic lupus erythematosus (SLE) is a chronic autoimmune inflammatory disease. Lupus nephritis (LN) is a major risk factor for mortality in SLE, and glomerular "full-house" immunofluorescence staining is a well-known characteristic of LN. However, some cases of non-lupus glomerulonephritis can also present with a "full-house" immunofluorescence pattern. We recently encountered a patient with full-house nephropathy (FHN) during adalimumab administration for Crohn's disease. IgA nephropathy or idiopathic FHN was diagnosed, and treatment with steroids was started, after which there was improvement in proteinuria. The prognosis of FHN has been reported to be poor; therefore, aggressive treatment is required for such patients.

Hydroxychloroquine use reduces mortality risk in systemic lupus erythematosus: A systematic review and meta-analysis of cohort studies.

Hydroxychloroquine (HCQ) is widely used in patients with systemic lupus erythematosus (SLE), but its effects on the mortality have not reached a definite conclusion. In this systematic review and meta-analysis, we aimed to assess whether HCQ use could reduce the risk of mortality in SLE patients. PubMed, Embase, Web of Science, and Cochrane database were searched from inception to April 17, 2022 without language restrictions to explore the relationship between HCQ use and SLE mortality. The relative risk (HR) was pooled using the STATA software. A total of 21 studies with a pooled patient population of 26,037 were included in the study, including 14 studies on the association between HCQ alone and mortality risk and seven studies on the association between HCQ/chloroquine (CQ) and mortality risk. The pooled findings suggested that HCQ significantly reduced the overall mortality risk of SLE (pooled HR 0.46, 95% CI 0.38-0.57, p < 0.001). In subgroup analysis of SLE complications, HCQ use also decreased the risk of death in SLE patients with renal (HR=0.43, 95% CI 0.26-0.70, p = 0.001) and cardiopulmonary involvement (HR=0.37, 95% CI= 0.25-0.54, p < 0.001). In addition, HCQ use was also protective against the risk of mortality in SLE patients in different regions, such as Asia (HR=0.46, 95% CI=0.33-0.64, p < 0.001), Europe (HR= 0.40, 95% CI = 0.22-0.71, p = 0.002), and America (HR=0.52, 95% CI= 0.42-0.64, p < 0.001). Our data suggested that HCQ use was associated with a reduced risk of mortality in patients with SLE.

91 Systemic lupus erythematosus and infectious complications: a pediatric case report

IntroductionInfection is one of the leading causes of morbidity and mortality in Systemic Lupus Erythematosus (SLE). Bacterial infections are the most frequent, followed by viral and fungal infections. Therapeutic factors as well as disease- and genetic related factors, all contribute to a lupus patient's increased susceptibility to infections.This report describes a case of a 12-year-old female who presented with features of systemic lupus erythematosus with inaugural infectious joint involvement.Methods and resultsAn 11- year- old girl, from Biskra (Algeria), from a non-consanguineous marriage and the 7th of eight children. She had no relevant past medical history. She was referred to our department for the diagnostic and therapeutic management of a suspected systemic lupus erythematosus.The girl had a fever with a malar erythema and oral ulcerations. Arthralgia of the right hip was reported. Laboratory tests showed a positive CRP (C-reactive protein) and a positive Procalcitonin. The immunological assessment revealed Anti DNA, Anti Sm and Anti RNP positive.Ultrasound of the hip showed a very large arthro-synovial cyst of the anterior face of the right hip of 07 cm compressing the psoas muscle. The cyst was drained and antibiotic therapy initiated. Hydroxychloroquine was started while waiting for the rest of the exploration.ConclusionJoint involvement in systemic lupus erythematosus is frequent but an infectious complication is possible even at the disease onset and should not be missed.No conflicts of interest to declare.

WITHDRAWN

SESSION TITLE: Critical Care of Immunocompromised Patients Case Posters SESSION TYPE: Case Report Posters PRESENTED ON: 10/19/2022 12:45 pm - 01:45 pm INTRODUCTION: Hyperpyrexia is defined as a core body temperature greater than 41.5 °C or 106.7°F. In modern times, hyperpyrexia is most prevalent in central nervous system hemorrhages. It can be seen in severe bacterial infections, though uncommon in modern times. Other more common causes of severe hyperthermia can include heat stroke and drug toxicity. This case presents a much less obvious cause of hyperpyrexia. CASE PRESENTATION: 27-year-old previously healthy female presents dysarthria and aphasia. Vitals revealed a temperature of 106.8 °F. Patient was admitted to ICU. Initial laboratory evaluation revealed renal failure, liver failure, rhabdomyolysis, troponin leak and hemolytic anemia. Nephrology started dialysis. MRI brain showed focal area of restricted diffusion involving the splenium of the corpus callosum. A CT chest/abdomen/pelvis showed no acute abnormalities. The patient's GCS deteriorated and was intubated. Rheumatology and hematology were consulted. High dose steroids were started. Due to high suspicion for an autoimmune process, the patient underwent plasmapheresis. Work-up revealed multiple positive autoimmune serologies. Extensive infectious workup was negative. the patient was extubated and kidneys began to recover. Renal biopsy revealed active focal and membranous lupus nephritis, ISN/RPS III + V. A diagnosis of severe lupus flare was made. Mycophenolate was initiated at this time. Steroids were tapered. The fever decreased but never resolved. She was then transferred to a tertiary center for biologics. DISCUSSION: Systemic Lupus Erythematosus is a relatively common autoimmune disease that involves multiple organ systems. Fever is a common manifestation of SLE and can occur in 36–86% of patients (1). According to the 2019 EULAR/ACR criteria, Fever is a low weighted diagnostic criterion for SLE. However, lupus flare fever is usually low-grade, rarely exceeding 102 F (2). The patient was found to be negative for infection, drug toxicity, heat stroke, intracranial bleed, hypothalamic infarct, or large vessel occlusion. It was therefore postulated that the biopsy proven acute SLE flare caused the hyperpyrexia. However, it is unclear if the entirety of the poly organ failure was caused by autoimmune reaction with organs themselves or by the hyperpyrexia. Only the Lupus Nephritis was confirmed by biopsy. Temperature of 38.5°C in non-infected patients result in increased mortality and adverse outcomes. (3). In this case, we presume that most of the patient's poly-organ dysfunction was either caused or perpetuated by hyperpyrexia. The full extent that the fever contributed to any specific organ dysfunction cannot be concretely demonstrated. CONCLUSIONS: This case demonstrates that in patients with hyperpyrexia or severe hyperthermia, autoimmune etiology should not be overlooked. This case presents a previously healthy, young female with hyperpyrexia caused solely by severe lupus flare. Reference #1: 1. Falagas ME, Manta KG, Betsi GI, Pappas G. (2007). Infection-related morbidity and mortality in patients with connective tissue diseases: a systematic review. Clin Rheumatol, 26: 663– 670. Reference #2: 2. Bernatsky S, Boivin JF, Joseph L, et al. (2006). Mortality in systemic lupus erythematosus. Arthritis Rheum, 54: 2550– 2557. Reference #3: 3. Edward Walter, Oliver R. Gibson. (2020). The efficacy of steroids in reducing morbidity and mortality from extreme hyperthermia and heatstroke—A systematic review. Pharmacol Res Perspect. 2020; 8:e00626 DISCLOSURES: No relevant relationships by Syed Akbarullah No relevant relationships by Sara Alleyasin No relevant relationships by Kory Cummings No relevant relationships by LLOYD Del Mundo No relevant relationships by Gerard DiChiara No relevant relationships by Jerome Hruska No relevant relationships by Amber McDonald No relevant relationships by Xenia Schneider No relevant relationships by Paul Stewart

Placental damage in pregnancies with systemic lupus erythematosus: A narrative review.

Systemic lupus erythematosus (SLE) is a chronic inflammatory autoimmune disease of unknown cause, which mainly affects women of childbearing age, especially between 15 and 55 years of age. During pregnancy, SLE is associated with a high risk of perinatal morbidity and mortality. Among the most frequent complications are spontaneous abortion, fetal death, prematurity, intrauterine Fetal growth restriction (FGR), and preeclampsia (PE). The pathophysiology underlying obstetric mortality and morbidity in SLE is still under investigation, but several studies in recent years have suggested that placental dysfunction may play a crucial role. Understanding this association will contribute to developing therapeutic options and improving patient management thus reducing the occurrence of adverse pregnancy outcomes in this group of women. In this review, we will focus on the relationship between SLE and placental insufficiency leading to adverse pregnancy outcomes.

Systemic Lupus Erythematosus and Atherosclerosis

Disorders likely ‘inflammatory’ in nature are known to be linked to accelerated atherosclerotic processes that increase the chances of cardiovascular disease. Systemic lupus erythematosus (SLE) is a well-known autoimmune disease for its ability to affect any organ and cause morbidity. One such major cause of morbidity and mortality in SLE is premature coronary heart disease. Inflammation is considered to be the main pathogenesis of atherosclerosis and an important risk factor for vascular disease. Many clinical trials and studies of epidemiological and pathogenesis-related factors revealed that there is a common link between the pathogenesis of autoimmune diseases such as SLE, causing inflammatory responses similar to those seen in atherosclerosis. In the following review article, we will describe how SLE, inflammation and its traditional risk factors, promotes atherosclerosis.

Short- and Long-Term Progression of Kidney Involvement in Systemic Lupus Erythematosus Patients with Low-Grade Proteinuria.

Lupus nephritis remains a common cause of morbidity and mortality in systemic lupus erythematosus (SLE). Current guidelines recommend performing a kidney biopsy at a urine protein-creatinine ratio of ≥0.5 g/g. However, cross-sectional studies reported a high prevalence of active histologic lupus nephritis lesions, and even chronic irreversible scarring, in patients with low-grade proteinuria. This study was initiated to assess disease progression in patients with SLE and low-grade proteinuria to identify risk factors for progression to overt proteinuria suggestive of clinical lupus nephritis. Patients with SLE who had an incident urinary protein-creatinine ratio of ≥0.2 and <0.5 g/g without known lupus nephritis were identified from the Einstein Rheumatic Disease Registry. Patients who developed a random urinary protein-creatinine ratio of ≥0.5 g/g with or without biopsy during the follow-up period were defined as "progressors." Patients who progressed to a urinary protein-creatinine ratio of ≥0.5 g/g within 2 years of developing a urinary protein-creatinine ratio of ≥0.2 and <0.5 g/g were defined as "fast progressors," a subgroup expected to benefit most from early biopsies and therapeutic interventions. Among 151 eligible patients with SLE and low-grade proteinuria at study entry, 76 (50%) progressed to a urinary protein-creatinine ratio of ≥0.5 g/g, of which 44 underwent a clinically indicated biopsy. The median (interquartile range) time from a urinary protein-creatinine ratio of ≥0.2 and <0.5 g/g to progression was 1.2 (0.3-3.0) years. Of the 20 biopsies performed in the first 2 years, 16 specimens showed active, treatable lupus nephritis. Low complement and shorter SLE duration at low-grade proteinuria onset were associated with progression to overt proteinuria across different analyses. Other associated factors included hypertension, diabetes mellitus, younger age, and the presence of hematuria. In this longitudinal cohort of patients with SLE and low-grade proteinuria at study entry, over half progressed to a urinary protein-creatinine ratio of ≥0.5 g/g in a short time period.

Ambulatory Blood Pressure Patterns in Children, Adolescents, and Young Adults With Childhood-Onset Systemic Lupus Erythematosus and Normal Office Blood Pressure.

Cardiovascular disease is a major cause of morbidity and mortality in systemic lupus erythematosus (SLE). Hypertension (HTN) and nondipping are modifiable risk factors for cardiovascular disease. Limited studies are available for childhood-onset SLE (cSLE). We aimed to assess ambulatory blood pressure monitoring (ABPM) pattern in cSLE patients with normal office blood pressure. This cross-sectional single-center study enrolled 25 patients with cSLE, normal office blood pressure and normal renal function. We performed 24-hour ABPM and echocardiography to assess end-organ damage. Descriptive statistics were obtained, and comparison of variables using Fisher exact test, t test, and Mann-Whitney U test was performed to identify potential risk factors for nondipping. Of the 25 patients, 22 were women; the median age was 18 years (interquartile range, 16-20 years). Median SLE duration was 4.2 years (interquartile range, 2.9-8.1 years); 18 patients had a history of lupus nephritis (LN). Four patients, 3 of whom had LN, had masked HTN. Fourteen patients (56%) were nondipping. The majority of patients without LN (71%) had a nondipping profile. Echocardiography was done for 15 patients. Left ventricular mass index, relative wall thickness, and ejection fraction were normal in all patients. Ambulatory blood pressure monitoring results led to changes in therapy in 5 patients. Our data provide evidence of high prevalence of nondipping and masked HTN in patients with cSLE, even in patients without LN. Identifying ABPM abnormalities in these patients could potentially improve outcomes.

Factors Associated With Candidiasis in Systemic Lupus Erythematosus Patients in Cipto Mangunkusumo National General Hospital: A Single-Center Case-Control Study.

Background: Infection has been a major cause of morbidity and mortality in systemic lupus erythematosus (SLE) patients. One of the infections encountered in SLE patients is candidiasis. Therefore, we aimed to conduct a case-control study to explore the risk factors associated with candidiasis in SLE patients in our center.Methods: Medical records of 20 SLE patients with fungal infection were obtained, and a case-control study was conducted with an age and sex-matched control group of 20 patients. Data were obtained from the Cipto Mangunkusumo National General Hospital. SLE confirmatory diagnosis was based on Systemic Lupus Erythematosus International Collaborating Clinics (SLICC) 2012 criteria. Patients with comorbidities of various chronic diseases (diabetes, HIV, and chronic kidney disease) were excluded. Statistical analysis was conducted using the Mann-Whitney U test with statistical significance defined as a p-value < 0.05.Result: Based on the analysis, a maximum corticosteroid dose of 24 (4-250) mg/day for the last one year was associated with the development of fungal infection (p = 0.047). Lower absolute lymphocyte count (748 cells/mm³ versus 1635 cells/mm³) was also associated with the occurrence of candidiasis in SLE patients (p = 0.008).Conclusion: Physicians should be aware that corticosteroid use in SLE patients may cause candidiasis. Monitoring of maximum corticosteroid dose and absolute lymphocyte count is important to help prevent candidiasis. Patients should also be educated regarding the risk of candidiasis from corticosteroid use.

Rising incidence and prevalence of systemic lupus erythematosus: a population-based study over four decades

ObjectivesTo determine the trends in incidence, prevalence and mortality of systemic lupus erythematosus (SLE) in a US population over four decades.MethodsWe identified all the patients with SLE in Olmsted County,...

Comparison of clinical presentation and outcome of childhood-onset and adulthood-onset of systemic lupus erythematosus among Indonesian patients.

The clinical presentation of childhood-onset systemic lupus erythematosus (SLE) is generally perceived to differ from that of adult-onset SLE. We aimed to compare the demographic and clinical manifestation between childhood-onset vs. adult-onset SLE in a cohort of Indonesian patients at tertiary care centers. This retrospective study included patients in the Hasan Sadikin Lupus Registry from 2008 until December 2017. The demographics, clinical presentations, and outcomes were compared between childhood-onset SLE (<18years old) (Group 1) and adult-onset SLE (≥18years old) (Group 2). Eight hundred seventy patients were involved into this study. The proportion of childhood-onset SLE was 20% (174 patients). The mean age of group 1 versus group 2 was 13.56 ± 3.04 vs 30.41 ± 8.54years. The following clinical manifestations at SLE diagnosis were significantly more common in childhood-onset than in adult-onset SLE patients: hematological disorder (p = 0.033) and arthritis (p = 0.006). While discoid rash (p = 0.036) and photosensitivity (p < 0.001) were significantly found higher in adult-onset SLE. Cyclophosphamide therapy was significantly more common to be used in childhood-onset (38.5% vs 21.0%, p = <0.001). However, frequency of mortality on follow-up tended to be higher in childhood-onset group (11.5% vs 7.0%, p = 0.208). Arthritis and hematologic involvements at SLE diagnosis were more prominent in childhood-onset compared to adult-onset patients, and mortality in childhood-onset SLE during follow-up relatively higher. This data may suggest the need for more aggressive management approach to childhood-onset patients with SLE.

Vascular Inflammation in Mouse Models of Systemic Lupus Erythematosus.

Vascular inflammation mediated by overly activated immune cells is a significant cause of morbidity and mortality in systemic lupus erythematosus (SLE). Several mouse models to study the pathogenesis of SLE are currently in use, many of which have different mechanisms of pathogenesis. The diversity of these models allows interrogation of different aspects of the disease pathogenesis. To better determine the mechanisms by which vascular inflammation occurs in SLE, and to assist future researchers in choosing the most appropriate mouse models to study cardiovascular complications in SLE, we suggest that direct comparisons of vascular inflammation should be conducted among different murine SLE models. We also propose the use of in vitro vascular assays to further investigate vascular inflammation processes prevalent among different murine SLE models.

Saving the kidneys in the lupus patient: Beyond immunosuppression, the need to collaborate across multiple disciplines

Systemic lupus erythematosus (SLE) is the classical systemic autoimmune disease with heterogeneous manifestations and unpredictable behaviour in which almost 70% may have a relapsing-remitting course, while 30% are divided equally between prolonged remission and persistently active disease [ [1] Tselios K. Gladman D.D. Touma Z. Su J. Anderson N. Urowitz M.B. Disease course patterns in systemic lupus erythematosus. Lupus. 2019; 28 ([Internet]JanAvailable from:): 114-122http://www.ncbi.nlm.nih.gov/pubmed/30526328 Crossref PubMed Scopus (28) Google Scholar ]. According to the mosaic of autoimmunity [ [2] Shoenfeld Y. Isenberg D.A. The mosaic of autoimmunity. Immunol Today. 1989; 10 ([Internet]AprAvailable from:): 123-126http://www.ncbi.nlm.nih.gov/pubmed/2665774 Abstract Full Text PDF PubMed Scopus (157) Google Scholar ], immune responses (innate and adaptative), as well as interaction of genes with environmental factors (ultraviolet radiation, smoking and drugs, such as, procainamide, hydralazine and anti-tumour necrosis factor agents) lead to numerous immunologic abnormalities that culminate in persistent immune responses against autologous nucleic acids. In this scenario, lupus nephritis (LN), the most frequent severe complication appears in 35% to 50% of patients in the first ten years of disease and contributes as a major cause for morbidity, rising medical expenses and mortality in SLE.

Twenty-year trends in all-cause mortality of patients with systemic lupus erythematosus in Mexico: Results from a nationwide health registry.

Regional variations in systemic lupus erythematosus (SLE) mortality may be due to different spectra of local environmental factors. The aim of this study was to assess mortality trends in adults with SLE using a nationwide health registry. Data came from the Dynamic Cubes of the General Direction of Health Information for 1998-2017 for mortality. In patients aged ≥15years, SLE as the principal cause of death was defined according to ICD-10 code M32 and was classified by sex and age. Joinpoint trend analyses of annual age-standardized mortality rates (ASMR) for SLE patients and non-SLE people were made. We identified 11449 SLE deaths and 9,989,874 non-SLE deaths. The SLE ASMR increased more than the non-SLE ASMR, with a 98.2% cumulative increase in the ratio of SLE to non-SLE deaths. Whereas the non-SLE ASMR remained relatively stable throughout the study period (overall and by sex), the SLE ASMR significantly increased between 1998 and 2009, non-significantly decreased between 2009 and 2013 and non-significantly increased thereafter. Both women and men had a large cumulative increase in the SLE ASMR/non-SLE ASMR ratio (73.9 and 191.3%, respectively). The Southeast region had the largest cumulative increases in the ratio of SLE to non-SLE ASMR (108.8%). Of the 11,449deaths, 445 (3.8%) were in geographical areas where ≥40% of the population is indigenous. SLE mortality rates have increased since 1998 and remain high compared with non-SLE mortality: significant sex and regional disparities persist.

Activity of Protein S-C4b Binding Protein and Total TFPI Levels in Egyptian SLE Patients: A Cross-Sectional Study

BACKGROUND: Systemic lupus erythematosus (SLE) is an immune disorder with alternating active and remission phases. Cardiovascular diseases and thrombosis are the major causes of mortality in SLE. The anticoagulant activity of Protein S (PS) is complemented by C4 binding protein (C4BP) and tissue factor pathway inhibitor (TFPI). AIM: This study aims to determine the extent of change in the levels of PS activity, C4BP, and total TFPI in active SLE in comparison to the SLE remission phase and their association with thrombosis during SLE flare. METHODS: The study included 180 Egyptian SLE patients who were classified into two groups: 100 SLE cases as the active group and 80 SLE cases as the remission group. The PS activity levels were processed on automated coagulation analyzers, whereas the C4BP and total TFPI levels were measured via enzyme-linked immunosorbent assay. RESULTS: The PS activity and C4BP levels were lower in the active SLE cases than in the remitted ones (p &lt; 0.05). The levels of PS activity and C4BP were revealed to be independent predictors of SELENA-SLEDAI flare scores. In active SLE cases, the PS activity and C4BP levels were rated as excellent and fair classifiers of thrombotic risk in SLE flare, respectively. The total TFPI levels showed no association with SLE activity or its thrombotic consequences. CONCLUSIONS: The levels of PS activity and C4BP act as important biomarkers for SLE activity. Both can be implanted as predictive tools for thrombosis during activity.

Glycosphingolipid Levels in Urine Extracellular Vesicles Enhance Prediction of Therapeutic Response in Lupus Nephritis.

The development of nephritis increases the risk of morbidity and mortality in systemic lupus erythematosus (SLE) patients. While standard induction therapies, such as mycophenolate mofetil (MMF) induce clinical remission (i.e., complete response) in approximately 50% of SLE patients with nephritis, many patients fail to respond. Therapeutic response is often not assessed until 6–12 months after beginning treatment. Those patients that fail to respond to treatment continue to accumulate organ damage, thus, there is a critical need to predict which patients will fail therapy before beginning treatment, allowing physicians to optimize therapy. Our previous studies demonstrated elevated urine, but not serum, glycosphingolipids (GSLs) in SLE patients with nephritis compared to SLE patients without nephritis, suggesting the urine GSLs were derived from the kidney. In this study, we measured the GSLs hexosylceramide and lactosylceramide in extracellular vesicles isolated from longitudinal urine samples of LN patients that were treated with MMF for 12 months. GSL levels were significantly elevated in the baseline samples (prior to treatment) of non-responders compared to complete responders. While a few other proteins measured in the whole urine were higher in non-responders at baseline, only GSLs demonstrated a significant ability to discriminate treatment response in lupus nephritis patients.

Proportionate Cardiovascular Mortality in Chronic Inflammatory Disease in Adults in the United States From 1999 to 2019.

Despite a rising prevalence of chronic inflammatory disease (CID), the recent trends in cardiovascular disease (CVD) mortality of patients with CID is scarce. In this study, we investigated patterns of CVD mortality in systemic lupus erythematosus (SLE), inflammatory bowel disease (IBD), and rheumatoid arthritis (RA) compared with the general population. We used the 1999 to 2019 multiple causes of death files from the national center for health statistics to analyze patterns and trends of proportionate CVD mortality in CID compared with the general population. We analyzed a total of 11,154 CVD deaths in IBD, 58,337 CVD deaths in RA, 6227 CVD deaths in SLE, and 17,826,871 CVD deaths in the general population. Between 1999 and 2019, we found that proportionate CVD mortality decreased significantly in the IBD group (25% to 16%), RA group (34% to 25%), and the general population (41% to 31%), but did not change for the SLE group (15% to 15%). Patients with SLE who died of CVD were approximately 10 years younger compared with CVD decedents with RA, IBD, or general population. The White population had higher proportionate CVD mortality than African American (IBD [19% vs 16%-18%] and SLE [14%-16% vs 12-14%], respectively). This study identifies current trends in CVD mortality in the CID population and elucidates current demographics in CVD mortality in CID. Although proportionate CVD mortality decreased in the general population, and in patients with RA and IBD, there was no change among patients with SLE. Further studies are needed to elucidate these differences.