Death In Systemic Lupus Erythematosus Research Articles

Prognostic value and predictors of the alteration of the diffusing capacity of the lungs for carbon monoxide in systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) is a systemic autoimmune disease characterized by heterogeneous manifestations and severity, with frequent lung involvement. Among pulmonary function tests (PFT), the measure of the diffusing capacity of the lungs for carbon monoxide (DLCO) is a noninvasive and sensitive tool assessing pulmonary microcirculation. Asymptomatic and isolated DLCO alteration has been frequently reported in SLE, but its clinical relevance has not been established. This retrospective study focused on 232 SLE patients fulfilling the 2019 EULAR/ACR classification criteria for SLE. Data were collected from the patient's medical record, including demographic, clinical, and immunological characteristics while DLCO was measured when performing PFT as part of routine patient follow-up. At the end of follow-up, DLCO alteration (<70% of predicted value) was measured at least once in 154 patients (66.4%), and was associated with a history of smoking as well as interstitial lung disease (ILD), but was also associated with renal and neurological involvement. History of smoking, detection of anti-nucleosome autoantibodies and clinical lymphadenopathy at diagnosis were independent predictors of DLCO alteration, while early cutaneous involvement with photosensitivity was a protective factor. DLCO alteration, at baseline or anytime during follow-up was predictive of admission in intensive care unit and/or of all-cause death, both mainly due to severe disease flares and premature cardiovascular complications. This study suggests a link between DLCO alteration and disease damage, potentially related to SLE vasculopathy, and prognostic value of DLCO on death or ICU admission in SLE.

A prognostic model for systemic lupus erythematosus-associated pulmonary arterial hypertension: CSTAR-PAH cohort study

BackgroundPulmonary arterial hypertension is a major cause of death in systemic lupus erythematosus, but there are no tools specialized for predicting survival in systemic lupus erythematosus-associated pulmonary arterial hypertension.Research questionTo develop a practical model for predicting long-term prognosis in patients with systemic lupus erythematosus-associated pulmonary arterial hypertension.MethodsA prognostic model was developed from a multicenter, longitudinal national cohort of consecutively evaluated patients with systemic lupus erythematosus-associated pulmonary arterial hypertension. The study was conducted between November 2006 and February 2020. All-cause death was defined as the endpoint. Cox regression and least absolute shrinkage and selection operators were used to fit the model. Internal validation of the model was assessed by discrimination and calibration using bootstrapping.ResultsOf 310 patients included in the study, 81 (26.1%) died within a median follow-up of 5.94 years (interquartile range 4.67–7.46). The final prognostic model included eight variables: modified World Health Organization functional class, 6-min walking distance, pulmonary vascular resistance, estimated glomerular filtration rate, thrombocytopenia, mild interstitial lung disease, N-terminal pro-brain natriuretic peptide/brain natriuretic peptide level, and direct bilirubin level. A 5-year death probability predictive algorithm was established and validated using the C-index (0.77) and a satisfactory calibration curve. Risk stratification was performed based on the predicted probability to improve clinical decision-making.ConclusionsThis new risk stratification model for systemic lupus erythematosus-associated pulmonary arterial hypertension may provide individualized prognostic probability using readily obtained clinical risk factors. External validation is required to demonstrate the accuracy of this model's predictions in diverse patient populations.

Mortality and causes of death in systemic lupus erythematosus in New Zealand: a population-based study.

This study aims to assess the mortality of systemic lupus erythematosus (SLE) patients and examine whether there are variations by subgroup. SLE patients from 2005 to 2021 were identified from the national administrative datasets. The underlying causes of death were examined. Standardized mortality ratio (SMR) was estimated to compare the relative rate of observed deaths in SLE patients with expected deaths in the general population. The hazard ratios (HR) and 95% confidence intervals (CI) of all-cause mortality and SLE specific mortality by ethnicity were estimated after adjustment for age using a Cox proportional hazards model. Of the 2802 patients included for analysis, 699 (24.9%) died with 209 (29.9%) SLE deaths. The age-standardized mortality rate of SLE was 0.29 per 100 000 for women and 0.05 for men. The mean age at death was 65.3 (17.1) years. Younger patients were more likely to have SLE as the underlying cause of death, from 78.9% for those under 20 years old to 18.7% for those aged 70-79 years. Compared with the general population, SLE patients were four times more likely to die (SMR: 4.0; 95% CI: 3.7, 4.3). Young patients had higher SMRs than older patients. Māori had worse all-cause mortality (HR: 1.72; 95% CI: 1.10, 2.67) and SLE specific mortality (HR: 2.60; 95% CI: 1.29, 5.24) than others. The outcomes of SLE in New Zealand were still very poor compared with the general population. Māori with SLE had worse survival than others. Further research is needed to identify the reasons for this disparity.

A Framework to Overcome Challenges in the Management of Infections in Patients with Systemic Lupus Erythematosus.

Infections remain one of the leading causes of death in systemic lupus erythematosus (SLE), despite awareness of factors contributing to increased susceptibility to infectious diseases in SLE. Clinicians report challenges and barriers when encountering infection in SLE as certain infections may mimic a lupus flare. There are no evidence-based practice guidelines in the management of fever in SLE, with suboptimal implementations of evidence-based benefits related to infectious disease control and/or prevention strategies in SLE. Vigilance in identifying an opportunistic infection must be stressed when confronted by a diagnostic challenge during a presentation with a febrile illness in SLE. A balanced approach must focus on management of infections in SLE, and reduction in the glucocorticoids dose, given the need to control lupus disease activity to avoid lupus related organ damage and mortality. Clinical judgement and application of biomarkers of lupus flares could reduce false positives and overdiagnosis and improve differentiation of infections from lupus flares. Further precision-based risk and screening measures must identify individuals who would benefit most from low dose immunosuppressive therapy, targeted immune therapy, and vaccination programs.

PREDICTION OF ATHEROSCLEROSIS DEVELOPMENT AND MODIFICATION OF ITS RISK FACTORS IN PATIENTS WITH SYSTEMIC LUPUS ERYTHEMATOSUS

Objective: Early-onset atherosclerosis with cardiovascular (CV) sequelae is the major cause of death in systemic lupus erythematosus (SLE); that cannot be explained only by traditional Framingham risk factors as SLE-related factors may also have an impact. The present study aimed to analyze the determinants of atherosclerosis development and risk factors modification by statin therapy in SLE patients. Design and method: This prospective randomized study included 100 SLE patients aged 40.9±1.4 years (90 females, 10 males) and 32 age-, sex-matched healthy controls. Brachial artery flow-mediated dilation (FMD), ultrasound of extracranial and peripheral arteries, and cardiac calcium scoring were used for the identification of atherosclerosis. 52 SLE patients with dyslipidemia were randomized to receive simvastatin 40 mg/day (n = 27) or placebo (n = 25). Lipid panel, FMD, and inflammatory markers were assessed at baseline and after 6 months. Results: The mean duration of SLE was 9.9±0.88 years. The disease activity index (SLEDAI) and SLE damage score (SLICC) were 11.8±0.6 and 1.85±0.13 points, respectively. In 66% of SLE patients, atherosclerosis was present in at least one vascular area which was 3.5 times more frequently compared with controls. Atherosclerosis of carotid arteries was detected in 41% of SLE patients, lower limb arteries, and coronary arteries in 58% and 39% of patients, respectively. FMD was significantly decreased in SLE patients compared to controls (7.95±0.49% vs 11.6±0.3%, p&lt;0.001). In multiple linear regression, age (p&lt;0.001), nephritis (p = 0.001), polyarthritis (p = 0.019), and Raynaud's syndrome (p = 0.045) were associated with low FMD. In binary logistic regression, independent determinants of the atherosclerosis development in SLE were age (p&lt;0.001), body mass index (BMI) (p = 0.001), anti-dsDNA (p = 0.023), FMD (p = 0.017) and malar rash (p = 0.02). After simvastatin treatment, the level of LDL-Cholesterol reduced by 42%, erythrocyte sedimentation rate, C-reactive protein, and interleukin-6 decreased by 34.7%, 51.1%, and 47.7% respectively, while FMD increased by 26.0% (p&lt;0.05); in the placebo group, there were no significant changes. Conclusions: The independent determinants of atherosclerosis in SLE patients were both traditional CV risk factors (age, BMI) and SLE-specific factors (anti-dsDNA, malar rash, FMD). In SLE patients with dyslipidemia, simvastatin may have not only lipid-lowering but also potentially anti-inflammatory benefits.

Mortality and causes of death in systemic lupus erythematosus revisited

Mortality and causes of death in systemic lupus erythematosus revisited

308 Machine learning to predict genetic variation and cardiovascular risk in Hispanic patients with Systemic lupus erythematosus

OBJECTIVES/GOALS: Cardiovascular disease (CVD) is the most common cause of death in systemic lupus erythematosus (SLE). Genome-wide association studies have identified single nucleotide polymorphisms linked with CVD risk, but the association with SLE is not well established. We aimed to determine associations using machine learning in a multi-ethnic lupus cohort. METHODS/STUDY POPULATION: We will use data from the established SLE cohort study named Genetic Profile Predicting the Phenotype (PROFILE). PROFILE was constituted in 1998 by combining existing cohorts at multiple sites which are also of defined ethnicity (Hispanics of Mexican ancestry and Puerto Rico, African American, and Caucasian). The cohort consists of 3,118 individuals and the database contains socioeconomic–demographic, clinical, laboratory, and genetic variables. Genetic data consist of 196,524 single nucleotide polymorphisms. To detect risk genes and predict an individual’s SLE risk will design a random forest classifier using SNP genotype data. Logistic regression models will be performed with CVD as the outcome, adjusted for age, sex, ethnicity, disease duration, and traditional and nontraditional risk factors for CVD. RESULTS/ANTICIPATED RESULTS: We expect to find several established and new susceptibility genes associated with CVD. DISCUSSION/SIGNIFICANCE: This approach offers an opportunity to characterize distinct genetic risk factors and the relationship of CVD with SLE. These data may be important in the identification of patients at high risk for such events and may allow the design of preventive strategies which may beneficially have an impact on the morbidity and mortality of SLE patients.

Primary aldosteronism: an unsuspected culprit of hypertension in systemic lupus erythematosus?

Primary aldosteronism (PA) is the most common endocrine cause of secondary hypertension and is associated with a high risk of cardiovascular disease in the general population. Patients suffering from systemic lupus erythematosus (SLE), a multisystem and multifactorial autoimmune disease, experience a high burden of hypertension and cardiovascular disease. Importantly, cardiovascular disease is one of the leading causes of death in SLE. Very limited evidence suggests an increased proportion of autoimmune diseases such as SLE in patients with PA. However, studies evaluating the prevalence of PA in the SLE population are lacking. Despite the potential for curative or targeted treatments, guidelines for the management of hypertension in SLE do not currently recommend testing for PA. This review highlights PA as a potentially over-looked secondary cause of hypertension in SLE, and offers future directions in research to improve the detection of this highly modifiable cardiovascular risk factor in the SLE population.

An Antiphospholipid Antibody Profile as a Biomarker for Thrombophilia in Systemic Lupus Erythematosus

Despite recent advances in treatment and significant improvements in prognosis, thrombosis remains the major cause of death in systemic lupus erythematosus (SLE). Antiphospholipid antibodies (aPL) are the main triggers of thrombosis in patients with SLE, with a frequency of approximately 30-40%. Lupus anticoagulant, anticardiolipin, and anti-β2-glycoprotein I antibodies, which are included in the criteria for antiphospholipid syndrome, and 'non-criteria' aPL such as anti-phosphatidylserine/prothrombin complex antibodies, are risk factors for thrombosis in patients with SLE. Multiple positivity for aPL is also associated with an increased risk of thrombosis, and scores calculated from aPL profiles can predict the risk of developing thrombosis. Although there is insufficient evidence for treatment, aPL-positive SLE patients should/may be treated with anticoagulants and/or low-dose aspirin as appropriate. This review summarises the evidence on the clinical significance of the aPL profile as a biomarker of thrombophilia in patients with SLE.

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.

Lupus nephritis as a specific clinical and immunological phenotype of systemic lupus erythematosus

Lupus nephritis (LN) is the leading cause of death in systemic lupus erythematosus (SLE), so its early detection and treatment is of utmost importance. Features of the onset, clinical signs, certain morphological classes, as well as more aggressive therapy make it possible to assign SLE with LN to a distinct disease phenotype.Objective: to characterize the clinical, immunological and morphological features of the SLE phenotype with a predominant kidney involvement based on a comparative analysis of patients with LN and without LN.Patients and methods. The study included 400 patients with SLE who met the 2012 SLICC criteria and were hospitalized to V.A. Nasonova Research Institute of Rheumatology from 2013 to 2021. The diagnosis of LN was established in 192 (48%) patients, of which in 82 (43%) it was confirmed by pathological study of kidney biopsy specimens (the SLE group with LN). In 208 (52%) patients, no kidney damage was observed, and they constituted the SLE group without LN.All patients underwent a standard examination with an assessment of disease activity according to the SLEDAI-2K index, irreversible changes in organs according to the SLICC damage index, immunological disorders, clinical and biochemical blood tests, urinalysis according to unified methods, glomerular filtration rate, as well as pathological examination of kidney biopsy specimens for confirmation of LN in the presence of an appropriate clinical picture. In patients of both groups, a comparative study of the main clinical, laboratory, immunological manifestations of SLE, the features of the disease onset, its first clinical signs, possible trigger factors, and the drugs used was carried out.Results and discussion. In the LN group, insolation was more likely to trigger the development of SLE than in the group without LN (respectively, in 26% and 13% of cases; p=0.007). In turn, SLE without kidney damage more often than SLE with LN debuted during pregnancy or after childbirth.The first signs of the disease in almost 40% of patients with LN were proteinuria and/or changes in urinary sediment, edema, increased blood pressure, the development of LN in some cases was preceded by polyarthritis or combined lesions of the skin and joints, but no later than 6 months, signs of kidney damage appeared. In the SLE group without LN, polyarthritis (in 33%), combined lesions of the skin and joints (in 26%), and Raynaud's syndrome (in 16%; p &lt;0.0001) were more often observed at the onset. In patients with LN, erythematous lesions of the facial skin ("butterfly", in 42%), serositis (exudative pleuritis — in 44%, pericarditis — in 46%, ascites and hydrothorax — in 5%; p&lt;0.0001), as well as hematological disorders such as anemia (in 63%), leukopenia (in 49%) and thrombocytopenia (in 42%) were present more frequently. With the development of LN, an acute course and high activity of the disease occurred significantly more often. In the study of immunological parameters in the group without LN, lupus anticoagulant (in 6%) and antibodies to SS-A/Ro and SS-B/La (in 18 and 9% of patients, respectively) were detected significantly more often, while in the LN group — hypocomplementemia (in 81%; p&lt;0.0001). Therapy also differed significantly: patients with LN received higher doses of glucocorticoids (p&lt;0.0001), mycophenolate mofetil, and cyclophosphamide.Conclusion. SLE with LN can be considered a distinct disease phenotype with a set of characteristics (clinical and laboratory parameters, response to therapy, prognosis) that distinguish it from other SLE variants.

Infections Associated with Systemic Lupus Erythematosus: Tackling Two Devils in the Deep Blue Sea

Systemic lupus erythematosus (SLE) is a quintessential systemic autoimmune disease with multiorgan involvement. Although its pathology has been elucidated and therapeutic advances are being made, infections remain the leading cause of death in SLE. In addition to immune abnormalities caused by SLE itself, the use of nonspecific glucocorticoids and immunosuppressants during treatment induces a state of immune suppression, increasing the risk of infection. Bacterial infections are the most common, but impaired cell-mediated immunity may also lead to the development of opportunistic infections, as well as viral and fungal infections. When treating SLE, caution is required for the development of infectious complications, such as latent tuberculosis, de novo hepatitis B, Pneumocystis jiroveci pneumonia (PJP), herpes zoster, and cytomegalovirus infection. Vaccination is recommended for vaccine-preventable diseases for appropriate management of infections. For other infectious diseases, such as tuberculosis, PJP, and disseminated herpes zoster, adequate treatment is necessary.

Brain-Reactive Antibodies are Potential Biomarkers for Evaluating Therapeutic Efficacy in NPSLE Patients.

PurposeNeuropsychiatric systemic lupus erythematosus (NPSLE) is the main cause of disability and death in systemic lupus erythematosus (SLE). It can cause cognitive impairment and organic brain syndrome. Brain-reactive antibodies, such as anti-DNA/anti-N-methyl-D-aspartate receptor (NMDAR) antibodies (DNRAbs), anti-microtubule-associated protein 2 (anti-MAP2) antibodies, and anti-glial fibrillary acidic protein (anti-GFAP) antibodies are thought to participate in the progression of NPSLE and thus considered potential diagnostic biomarkers, but whether they can be used for evaluating therapeutic efficacy in NPSLE is unknown.Patients and methodsOverall, 17 NPSLE patients and 10 non-SLE controls were included in this study. All the patients were treated with glucocorticoid (GC) pulse therapy. Serum and cerebrospinal fluid (CSF) concentrations of DNRAbs and anti-MAP2 and anti-GFAP antibodies were measured using enzyme-linked immunosorbent assay. The differences between the CSF concentrations of these antibodies in NPSLE patients before and after GC pulse therapy were analyzed.ResultsCSF concentrations of DNRAbs and anti-MAP2 and anti-GFAP antibodies were significantly higher in NPSLE patients compared to the non-SLE controls. Among the patients, CSF concentration of DNRAbs was significantly higher in the patients with acute confusional state (ACS) than in those with non-ACS diffuse NPSLE or focal NPSLE. Additionally, CSF concentration of DNRAbs was significantly correlated with QIgG (r=0.4884, P=0.0467) and IgG index (r=0.5319, P=0.0280) in NPSLE patients. Moreover, CSF concentrations of DNRAbs, anti-MAP2, and anti-GFAP antibodies and QIgG were significantly decreased after GC pulse therapy in NPSLE patients.ConclusionThese results indicate that CSF DNRAbs and anti-MAP2 and anti-GFAP antibodies are potential biomarkers for evaluating therapeutic efficacy in NPSLE.

Epidemiology of interstitial lung disease in systemic lupus erythematosus in France: A nation-wide population-based study over 10 years.

Background and objectiveData regarding interstitial lung disease (ILD) in the setting of systemic lupus erythematosus (SLE) are limited. We used a nationwide database to determine the incidence and the prevalence of ILD in SLE.MethodsCharacteristics of all SLE inpatients admitted between 2011 and 2012 in France were analysed through the French medico‐administrative database. Features associated with the presence of ILD were studied. Cox hazard model was used to measure the impact of ILD on survival from the first stay to 2020. The incidence of ILD in SLE was estimated by analysing the onset of ILD from 2013 to 2020 in SLE patients who had no evidence of ILD in 2013.ResultsBetween 2011 and 2012, 10,460 SLE patients had at least one hospital stay and could be traced until 2020. Among them, 134 (1.2%) had an ILD diagnosed at baseline. The frequency of ILD in SLE was higher in patients who had an associated autoimmune disease such as Sjögren's syndrome or systemic sclerosis (29.9% vs. 5.9%, p < 0.0001). ILD was associated with an increased risk of death in SLE in the multivariable analysis (hazard ratio [95% CI] 1.992 [1.420–2.794]; p < 0.0001). Among the 31,029 SLE patients with no evidence of ILD at baseline, ILD occurred in 795 (2.6%) between 2013 and 2020. The incidence rate of ILD in SLE was 10.26 for 1000 patient‐years [95% CI: 10.24–10.28].ConclusionIn SLE, ILD is exceedingly rare, often associated with another systemic autoimmune disorder and appears as a major risk factor for death.

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.

Antimalarial treatment and minimizing prednisolone are associated with lower risk of infection in SLE: a 24-month prospective cohort study.

Infections are a major cause of morbidity and death in systemic lupus erythematosus (SLE). Perfecting the understanding of contributors to infection burden in SLE is pivotal to improve management and outcomes. This study aims to identify clinical predictors of infection in SLE. We conducted a prospective cohort study at a referral SLE clinic. Infections were identified at each visit and categorized as (a) any type, (b) serious, (c) non-serious, and (d) bacterial. Survival analysis followed by multivariate Cox regression with an estimation of hazard ratios (HR) with 95% confidence intervals (95%CI) was performed. We included 259 patients during a mean follow-up of 23.3 ± 5.7months. The incidence rate of infection of any type was 59.3 cases per 100 patient-years. Multivariate Cox models showed that (a) prednisolone ≥ 7.5mg/day (HR = 1.95, 95%CI 1.26-3.03) and female gender (HR = 2.08, 95%CI 1.12-3.86) were associated with higher risk of infection of any type; (b) prednisolone ≥ 10mg/day was associated with higher (HR = 4.32, 95%CI 1.39-13.40), and antimalarials with lower risk (HR = 0.18, 95%CI 0.06-0.51) of serious infection; (c) female gender (HR = 1.92, 95%CI 1.04-3.57) and prednisolone ≥ 7.5mg/day (HR = 1.89, 95%CI 1.21-2.96) were associated with higher risk of non-serious infection; (d) antimalarials were associated with lower (HR = 0.49, 95%CI 0.26-0.93) and female gender (HR = 5.12; 95%CI 1.62-16.18) with higher risk of bacterial infection. The risk of infection was higher in females in this young, well-controlled, low-comorbidity SLE cohort. Antimalarials were associated with lower and prednisolone ≥ 7.5mg with higher risk of infection. Key Points • Lupus patients treated with prednisolone ≥ 7.5mg/day were 89% more likely to present infections. • Lupus patients receiving prednisolone ≥ 10mg/day were four times more likely to present serious infections. • Lupus patients receiving antimalarials were 82% less likely to present serious infections.

Early Diagnosis of Neuropsychiatric Systemic Lupus Erythematosus by Deep Learning Enhanced Magnetic Resonance Spectroscopy

The neuropsychiatric systemic lupus erythematosus (NPSLE) has higher disability and mortality rates, which is one of the main causes of death in systemic lupus erythematosus (SLE) patients. Magnetic resonance spectroscopy (MRS) can detect the changes of metabolites in different intracranial areas in vivo in patients with SLE, so as to provide evidence for the early diagnosis of NPSLE. Different from the conventional single-voxel MRS, which can only screen one brain region with one metabolic change, we simultaneously detect 13 kinds of intracranial metabolic changes in nine brain regions by multivoxel proton MRS (MVS). We use a recursive feature elimination algorithm to select the most related metabolites for better identifying NPSLE. To accurately diagnosis NPSLE by these intracranial metabolites, we train a support vector machine deep stacked network (SVM-DSN) for quantitative analysis of these metabolites. Comparing with the conventional statistic method, which is about 70% of accuracy, the proposed model achieves 97.5% of accuracy for NPSLE diagnosis. We conclude the trained SVM-DSN can effectively analyze the metabolites obtained by multivoxel proton MRS for NPSLE diagnosis, which may help to early diagnosis and intervention of NPSLE, and alleviate the bias of manual screening.

Effects of BAFF Neutralization on Atherosclerosis Associated With Systemic Lupus Erythematosus.

Cardiovascular disease (CVD) is the leading cause of death in systemic lupus erythematosus (SLE). B cells play a key role in the pathogenesis of lupus, and anti-BAFF therapy has been approved for use in SLE. Since mature B cells also promote atherosclerosis, we undertook this study to evaluate, in a mouse model and in SLE patients, whether BAFF neutralization has an atheroprotective effect in SLE. The effect of BAFF on atherosclerosis associated with lupus was investigated in the atherosclerosis/lupus-prone apolipoprotein E-knockout D227K mouse model and in a cohort of SLE patients. Mice were treated with a blocking anti-BAFF monoclonal antibody (mAb), while fed a standard chow diet. Carotid plaque and carotid intima-media thickness were assessed by ultrasound at baseline and during follow-up in SLE patients who were asymptomatic for CVD. Anti-BAFF mAb in ApoE-/- D227K mice induced B cell depletion, efficiently treated lupus, and improved atherosclerosis lesions (21% decrease; P = 0.007) in mice with low plasma cholesterol levels but worsened the lesions (17% increase; P = 0.06) in mice with high cholesterol levels. The atheroprotective effect of the BAFF-BAFF receptor signaling inhibition on B cells was counterbalanced by the proatherogenic effect of the BAFF-TACI signaling inhibition on macrophages. In SLE patients, blood BAFF levels were associated with subclinical atherosclerosis (r = 0.26, P = 0.03). Anti-BAFF mAb treatment had a differential effect on the intima-media thickness progression in SLE patients depending on body mass index. Depending on the balance between lipid-induced and B cell-induced proatherogenic conditions, anti-BAFF could be detrimental or beneficial, respectively, to atherosclerosis development in SLE.

Дебют системной красной волчанки у мужчины пожилого возраста

Systemic lupus erythematosus (SLE) is a chronic autoimmune inflammatory disorder with a wide range of clinical and laboratory manifestations which occurs predominantly in young women. The most common age of onset is 20–30 years. However, SLE occasionally occurs after the age of 50 years. The clinical signs of SLE are different from those of a typical disease. As a result, a separate form, SLE in the elderly, was identified. SLE in the elderly often manifests as weight loss, fever, myalgia, arthralgia, pulmonary lesions, serositis, cardiovascular, hemolytic, and hematological disorders. The clinical course of late-onset SLE is usually favorable compared to classic SLE. However, poor survival is reported in these patients compared to early-onset SLE. The causes of death in late-onset SLE are infectious and vascular complications, the adverse effects of hormonal and cytostatic therapies, hematological and comorbid disorders. The authors describe a 67-year-old man with late-onset SLE. A set of tests verified the diagnosis. Efferent treatment was recommended.KEYWORDS: late-onset SLE, pericarditis, pleuritis, serositis, Rowell syndrome, efferent therapy, plasmapheresis.FOR CITATION: Aganov D.S., Toporkov M.M., Svintsitskaya I.S. et al. Onset of systemic lupus erythematosus in an elderly man. Russian Medical Inquiry. 2020;4(8):525–528. DOI: 10.32364/2587-6821-2020-4-8-525-528.

Mortalidad y causas de muerte en pacientes con lupus eritematoso sistémico

Introduction: Systemic Lupus Erythematosus (SLE) is an autoimmune disease characterized by chronic systemic inflammation. Causes of death in SLE have a bimodal pattern: during the first 5 years, infections and disease activity prevail, whereas beyond 5 years cardiovascular complications and accumulated organ damage are the main causes. Objective: To determine causes of death in adult patients diagnosed with SLE admitted to the Department of internal Medicine of the National Hospital from January 2010 to December 2018. Materials and methods: Retrospective, observational, descriptive, cross sectional study of adult patients admitted to the Department of internal Medicine and the Emergency Depart- ment of the National Hospital of Itaugua, from 2010 to 2018. Out of 585 patients with SLE admitted during this period, 63 died. 25 patients were included and 38 were excluded due to incomplete studies. SLE diagnosis was made according to SLiCC criteria. The variables analyzed were demographic, clinical, laboratory, causes of death, mortality. Data was analyzed with descriptive statistics. Frequencies and proportions were used for qualitative variables, means and standard deviation for continuous variables. Results: 25 patients were included, 18 were women (72%) and 7 were men (28%). Mean age was 30±15.5 (18-77). 40% (n=10) came from Central Department and 60% (n=15) were from the countryside. 70.8% of the patients had a disease duration between 2 and 5 years (n=18). 92% had been previously diagnosed with SLE. Regarding disease activity, patients presented with nephritis (76%), alveolar hemorrhage (12%), pneumonitis (4%), neurolupus (4%) and CNS vasculitis (4%). On the subject of infections, the following were found: nosocomial pneumonia (45%), community acquired pneumonia (8%), strongyloides hyperinfection (4%), disseminated cryptococcosis (4%). The main causes of death were infections (44%), followed by disease activity (36%) and causes not related to SLE (20%; severe mitral and aortic insufficiency, acute pulmonary edema secondary to chronic kidney disease as complication of lupus nephritis, hemorrhagic stroke). 100% received corticosteroids and 60% immunosuppressants. Conclusion: Mortality was 10.8%, the main causes of death were infections followed by disease activity.