Systemic Lupus Erythematosus Disease Activity Index-2K Research Articles

Predicting systemic lupus erithematosus disease activity index 2000 (Sledai 2k) score after pulse dose metyl prednisolone in severe systemic lupus erithematosus

Background: Systemic lupus erythematosus (SLE) is a chronic multisystem rheumatic disease characterized by developing autoantibodies against nucleus antigen. It has a broad range of clinical symptoms and the potential to affect nearly all organs and tissues. Pulse dosage methylprednisolone (MEP) is the immunosuppression modality for life-threatening or organ-threatening SLE. However, it is challenging to estimate the MEP response rate. We combine clinical symptoms, routine laboratory examinations, and more specific protein examinations such as soluble B-cell activating factor (sBAFF), B-cell activating factor receptor (BAFF-R), and Interferon gamma-induced protein 10 (IP-10) to develop a formula that can predict the SLE Disease Activity Index 2K (SLEDAI 2K) score following a pulse dosage of methylprednisolone. Methods: In a prospective cohort study, patients with severe SLE with a SLEDAI 2K score of 12 or Lupus nephritis class III or IV according to WHO criteria were given methylprednisolone 500 mg/day for three consecutive days. Enzyme-linked immunosorbent assay (ELISA) tested blood samples for soluble (s) BAFF, IP 10, and flow cytometry for BAFF-R, CD-19. The SLEDAI 2K score was reevaluated after a pulse dose of methylprednisolone was administered. All statistical analyses were conducted using the Rstudio program. Results: Overall, 80 patients were included. Multivariate multiple regression analysis revealed that urine protein creatinine ratio (UPCR) (x1), CD19 percentage (x2), serum BAFF (x3), vasculitis (x4), and rash (x5) taken before MEP pulse were predictors for SLEDAI 2k score after pulse dose methylprednisolone in severe SLE with the formula 13.41+ (0.0008542 * x1) + -0.1829338 * x2) + (0.0008776 * x3) + (7.1801728 * x4) + (7.5429676 * x6). Conclusions: Formula to predict SLEDAI 2k score after MEP pulse was 13.41+ (0.0008542 * x1) + -0.1829338 * x2) + (0.0008776 * x3) + (7.1801728 * x4) + (7.5429676 * x6). Further validation is needed to be used in clinical practice.

Belimumab versus anifrolumab in adults with systemic lupus erythematosus: an indirect comparison of clinical response at 52 weeks

ObjectiveTo generate comparative efficacy evidence of belimumab versus anifrolumab in SLE that can inform treatment practices.MethodsThe SLE Responder Index (SRI)-4 response at 52 weeks of belimumab versus anifrolumab was evaluated...

Serum levels and significance of soluble B-cell maturation antigen in childhood-onset systemic lupus erythematosus with renal involvement.

The aim of this study was to investigate serum levels of soluble B-cell maturation antigen (sBCMA) in childhood-onset systemic lupus erythematous (cSLE) patients with renal involvement, and to elucidate their association with clinical characteristics. 116 cases of cSLE patients with renal involvement (84 females and 32 males; median age 11.6 (10.1, 12.9) years) hospitalized in Department of Pediatric Nephrology and Rheumatology, the First Affiliated Hospital, Sun Yat-sen University and 31 healthy controls (HCs) were enrolled. Serum concentrations of sBCMA were determined using enzyme-linked immunosorbent assay (ELISA). Clinical and laboratory information of cSLE patients were retrospectively analyzed. Serum sBCMA levels were significantly increased in primary cSLE when compared with treated cSLE patients and HCs, whereas there was no significant difference between treated cSLE patients and HCs. Patients with high disease activity displayed higher serum sBCMA levels compared with those with no or mild to moderate disease activity. Positive correlation was observed between serum sBCMA levels and systemic lupus erythematosus disease activity index-2K (SLEDAI-2K), antinuclear antibody titers, anti-double-stranded DNA titers, erythrocyte sedimentation rate, and immunoglobulin G levels, while sBCMA levels were negatively correlated with blood white blood cell count, hemoglobin, platelet count, complement C3 and C4 levels. Serum sBCMA levels decreased as disease ameliorated after treatments among 11 cases with follow-up examinations. In cSLE patients with renal involvement, serum sBCMA levels correlated significantly with disease activity, immunological, and hematological parameters, but not with renal parameters. Our results suggest the potential and significance of serum sBCMA as a biomarker in cSLE patients.

Impact of depression on quality of life in systemic lupus erythematosus patients

BackgroundDepression is common in systemic lupus erythematosus (SLE) and is an unmeasured risk factor, yet its symptoms can be neglected in standard disease evaluations. The purpose of this study was to assess the frequency and the impact of depression on quality of life in SLE patients. We recruited 32 patients with SLE and 15 healthy control volunteers in the study. The following investigations were undertaken in each patient: clinical and rheumatologic assessment, SLE Disease Activity Index-2k (SLEDAI-2k), Beck Depression Inventory (BDI), Short-Form Health Survey (SF-36) questionnaire, and routine laboratory tests.ResultsThere was a high percentage of depression (46.9%) in the SLE patients. Regarding quality of life (SF-36), there were significant affection of the physical and mental composite summary domains (PCS and MCS) scores in lupus patients compared with controls (P < 0.000 for both) with the same significant in depressed compared with non-depressed patients. SF-36 subscales (physical function, limit emotional, emotional wellbeing, and social function) were significantly affected in depressed lupus patients compared with non-depressed patients. There was a significant negative correlation between the score of MCS domain of SF-36 with BDI (P < 0.000) while positive correlation between SLEDAI score with depression score. In contrast, there were no significant correlations between MCS or PCS with age, duration of illness, or SLEDAI-2K.ConclusionsDepression is common in SLE patients and had a negative impact on quality of life particularly on MCS domain and positive correlation with disease severity score.Trial registrationThis study was registered on clinical trial with registration number: NCT03165682 https://clinicaltrials.gov/ct2/show/NCT03165682 on 24 May 2017.

CD14 (C-159T) polymorphism is associated with increased susceptibility to SLE, and plasma levels of soluble CD14 is a novel biomarker of disease activity: A hospital-based case-control study.

Cluster of differentiation 14 (CD14) plays a crucial role in the innate immune response of the host in protection against various pathogens. The importance of soluble CD14 in autoimmune disorders has been described in different populations. However, the role of sCD14 in systemic lupus erythematosus (SLE) is poorly understood. Further, the association of functional variants at the promoter region of the CD14 gene (-159 C > T) with susceptibility to SLE or disease severity needs to be defined. Two hundred female SLE patients diagnosed on systemic lupus international collaborating clinics (SLICC) classification criteria and age, sex, matched healthy controls were enrolled in the present study. Polymerase chain reaction- restriction fragment length polymorphism (PCR-RFLP) method was used to genotype CD14 (C-159 T) polymorphism. Plasma levels of IFN-α, TNF-α, and sCD14 were quantified by enzyme-linked immunosorbent assay (ELISA). Prevalence of mutant genotypes (CT and TT) and minor allele (T) of CD14 (C-159T) polymorphism was significantly higher in SLE cases compared to healthy controls (CT: P < 0.0001; OR = 3.26, TT:P < 0.0001; OR = 3.39; T:P = 0.0009, OR = 1.62). Further, lupus nephritis patients had a higher prevalence of homozygous mutants (TT) and mutant allele (T)(TT: P = 0.0002, OR = 8.07; T: P = 0.001, OR = 1.32). SLE patients displayed significantly increased plasma sCD14, TNF-α, and IFN-α levels in comparison to healthy controls. These cytokines were significantly elevated in patients of lupus nephritis compared to those without kidney involvement. Interestingly, sCD14 levels correlated positively with SLE disease activity index-2K (SLEDAI-2K) scores and 24 hours proteinuria. CD14 (C-159T) polymorphism is associated with an increased predisposition to the development of SLE and lupus nephritis: sCD14 is a promising novel biomarker for assessing disease activity and lupus nephritis.

Incidence of psoriasis in patients with active systemic lupus erythematosus

Introduction: Concomitant onset of active systemic lupus erythematosus (SLE) and psoriasis is rare; however, due to the common skin involvement in both and exacerbation of psoriasis with antimalarial medications used for SLE, it is crucial to investigate the concomitant occurrence of both diseases. Objectives: The present study aims to investigate the incidence of psoriasis in people with active SLE. Patients and Methods: The present cross-sectional study was performed on 148 patients with active SLE from 2015 to 2017. Lupus activity was evaluated based on systemic lupus erythematosus disease activity index-2K (SLEDAI-2K), and then, the incidence of psoriasis in patients was investigated. Patients’ demographic information was recorded in the relevant checklist. Results: The mean score of SLEDAI-2K was 14.10±19.77. The duration of SLE was significantly associated with the SLEDAI-2K score (P=0.001, r = 0.294). Psoriasis incidence was remarkably correlated with the gender of patients with SLE (P=0.019). An insignificant association was found between the coincidences of SLE with psoriasis considering the factors, including age, SLE duration, and mean SLEDAI-2K score. Conclusion: Patients’ mean age, SLE duration, and disease activity score were not correlated with concomitant psoriasis among SLE patients; however, we found that males were more prone to concomitant SLE and psoriasis.

Age at diagnosis and health-related quality of life are associated with fatigue in systemic lupus erythematosus patients: Data from the Almenara Lupus Cohort.

To define the factors associated with fatigue in Mestizo patients with Systemic Lupus Erythematosus (SLE). This is a cross-sectional study of SLE patients from a single center cohort. Visits were performed every six months. For these analyses, the first visit between October 2017 and December 2018 was included. Demographic and clinical characteristics as well as treatment were recorded at every visit. Fatigue was ascertained with the Functional Assessment of Chronic Illness Therapy-Fatigue (FACIT-FT), Health-Related Quality of Life (HRQoL) with the LupusQoL, disease activity with the Systemic Lupus Erythematosus Disease Activity Index -2 K (SLEDAI-2K), and damage with the Systemic Lupus International Collaborating Clinics (SLICC)/American College of Rheumatology damage index (SDI). Prednisone use was recorded as current daily dose. Immunosuppressive drugs and antimalarial use were recorded as current, past or never. Univariable and multivariable analyses were performed using linear regression models. For the multivariable analyses, model selection followed a backward elimination procedure. Two hundred and twenty-six patients were evaluated. The mean (SD) age at diagnosis was 35.6 (13.1) years, 211 (93.4%) were female; and disease duration was 11.0 (7.3) years. The mean SLEDAI and SDI were 2.4 (3.5) and 1.3 (1.5), respectively. The mean FACIT-FT was 33.1 (10.8). On the multivariable analysis, age at diagnosis and some domains of HRQoL (physical health, emotional health and fatigue) remained associated. Age at diagnosis is negatively associated with fatigue whereas HRQoL domains like physical health, emotional health and fatigue are positively associated with fatigue.

FRI0178 PREDICTIVE FACTORS FOR POOR SLEEP QUALITY IN SYSTEMIC LUPUS ERYTHEMATOSUS

Background:Poor sleep quality is common in systemic lupus erythematosus (SLE) and could contribute to fatigue, which is regarded as one of the most disabling symptoms in SLE. The Pittsburgh sleep quality index (PSQI) is a validated self-administered questionnaire that measures sleep quality over the previous month.1Objectives:The aim of this study was to analyse the relationship of sleep quality with several variables including depression, anxiety, pain, disease activity, fatigue and functional disability in patients with SLE. A further aim was to establish the prevalence of poor sleep quality in SLE.Methods:A cohort cross-sectional study was carried out including 92 SLE patients who fulfilled the SLICC classification criteria for SLE and who provided informed consent for participation. The patients were interviewed and they were asked to fill in questionnaires including PSQI, Fatigue Severity Scale (FSS), Hospital Anxiety and Depression Scale (HADS), visual analogue scale (VAS) for pain and modified Health Assessment Questionnaire (mHAQ). Blood and urine tests enabled the calculation of SLE disease activity index-2K (SLEDAI-2K). The study was approved by the University Research Ethics Committee.Results:92.4% of the cohort studied were females, and the mean age was 46.9 years (range 19-79 years). 55.4% were noted to have poor quality sleep (PSQI &gt;5), and the median PSQI was 6 (range 0-18). Sleep quality measured by PSQI, had a significant correlation with SLEDAI-2K (R=0.254, p=0.014), VAS pain (R=0.515, p&lt;0.001), HADS-D (R=0.605, p&lt;0.001), HADS-A (R=0.375, p&lt;0.001), estimated glomerular filtration rate (eGFR) (R=-0.211, p=0.044), FSS (R=0.551, p&lt;0.001) and mHAQ (R=0.559, p&lt;0.001). ANCOVA analysis showed that PSQI was significantly dependant on VAS pain (p&lt;0.001), HADS-D (p&lt;0.001) and eGFR (p=0.003).Conclusion:Poor sleep quality is highly prevalent in SLE patients. This study has shown that the strongest predictive factors for poor sleep quality are pain, depression and impaired renal function. Since poor sleep quality is significantly related to fatigue and functional disability, its identification and management is important for patients’ wellbeing.

Evaluation of Lupus Low Disease Activity: Characteristics, Predictors, and Association with Disease Damage: A Retrospective Cohort from Two Tertiary Centers in Egypt

Abstract Background: Remission in systemic lupus erythematosus (SLE) is seldom achieved; making Lupus Low Disease Activity (LLDA) an alternative yet promising target. Aim of Study: The aim of this study was to evaluate the prevalence of remission and LLDA achieved, and the charac-teristics and predictors of LLDA, and its potential association with disease damage. Patients and Methods: The medical records of 243 patients fulfilling the 2012 Systemic Lupus Collaborating Clinics (SLICC) classification criteria for SLE and managed at Cairo and Ainshams Universities from January to December 2019 were viewed. Remission was categorized to: (i) Complete remission off glucocorticoid (GC) and Systemic Lupus Ery-thematosus Disease Activity Index-2K (SLEDAI-2K) = zero (antimalarial only); (ii) Clinical remission off GC with sero-logic activity (SKLEDAI-2K £4); (iii) Clinical remission on GC £5mg/day (SLEDAI-2K score £ 4 and serologic activity). LLDA was defined as SLEDAI-2K £4 in the absence of major organ involvement, GC dosage £7.5mg/day. Disease damage was assessed through the Systemic Lupus International Col-laborating Clinics/American College of Rheumatology Damage Index (SDI). Results: Seventy two (29.6%) patients achieved LLDA at the last visit; whereas 142 (58.4%) patients had a SLEDAI-2K score >4 and/or received a GC dosage of >7.5mg. One (0.4%) and 28 (11.5%) patients achieved clinical remission off and on GC, respectively. None of the patients achieved complete clinical and serologic complete remission. Patients achieving LLDA had an older age of onset compared to those with higher disease activity (p=0.003), and a lower prevalence of fever (p=0.009), weight loss (p=0.07), cutaneous vasculitis (p=0.002), serositis (p=0.006), nephritis (p=0.02), a lower median SDI score, and lower prevalence of developing severe damage (SDI ³3) (p=0.04). Predictors of LLDA were an older age of onset [p=0.006 (OR=1.05; 95% CI=1.01-1.09)] and weight loss [p=0.009 (OR=5; 95% CI=1.9-16.5)]; whereas patients with LLDA were less likely to have cutaneous vas-culitis [p=0.01 (OR=0.2; 95% CI=0.06-0.7)] or pleurisy and/or pleural effusion [p=0.001 (OR=0.2; 95% CI=0.1-0.5)].Conclusion: Achieving remission was substantially low. Lupus Low Disease Activity (29.6%) was associated with a higher age of onset, several distinct clinical characteristics, and lower damage.

P171 Factors determining sleep quality in SLE

Abstract Background Poor sleep quality is common in systemic lupus erythematosus (SLE) and could contribute to fatigue, which is regarded as one of the most disabling symptoms in SLE. The Pittsburgh sleep quality index (PSQI) is a validated self-administered questionnaire that measures sleep quality over the previous month. The aim of this study was to analyse the relationship of sleep quality, measured by PSQI, with several variables including depression, anxiety, pain, disease activity, fatigue and functional disability in patients with SLE. A further aim was to establish the prevalence of poor sleep quality in SLE. Methods A cohort cross-sectional study was carried out including 92 SLE patients who fulfilled the SLICC classification criteria for SLE and who provided informed consent for participation. The patients were interviewed, and they were asked to fill in questionnaires including PSQI, Fatigue Severity Scale (FSS), Hospital Anxiety and Depression Scale (HADS), visual analogue scale (VAS) for pain and modified Health Assessment Questionnaire (mHAQ). Blood and urine tests enabled the calculation of SLE disease activity index-2K (SLEDAI-2K). The study was approved by the University Research Ethics Committee. Results 92.4% of the cohort studied were females, and the mean age was 46.9 years (range 19-79 years). 55.4% were noted to have poor quality sleep (PSQI &amp;gt;5), and the median PSQI was 6 (range 0-18). Sleep quality measured by PSQI, had a significant correlation with SLEDAI-2K (R = 0.254, p = 0.014), VAS pain (R = 0.515, p &amp;lt; 0.001), HADS-D (R = 0.605, p &amp;lt; 0.001), HADS-A (R = 0.375, p &amp;lt; 0.001), estimated glomerular filtration rate (eGFR) (R=-0.211, p = 0.044), FSS (R = 0.551, p &amp;lt; 0.001) and mHAQ (R = 0.559, p &amp;lt; 0.001). ANCOVA analysis showed that PSQI was significantly dependant on VAS pain (p &amp;lt; 0.001), HADS-D (p &amp;lt; 0.001) and eGFR (p = 0.003). Conclusion Poor sleep quality is highly prevalent in SLE patients. This study has shown that the strongest predictive factors for poor sleep quality are pain, depression and impaired renal function. Since poor sleep quality is significantly related to fatigue and functional disability, its identification and management is important for patients’ wellbeing. Disclosures R. Magro None. L. Camilleri None. A.A. Borg None.

Çocukluk çağı başlangıçlı sistemik lupus eritematozus hastalarında avasküler nekrozis sıklığı

Purpose: Avascular necrosis (AVN) is a debilitating complication of juvenile systemic lupus erythematosus (jSLE). The aim of this study was to evaluate the frequency and clinical characteristics of patients with AVN in jSLE from a single center. Material and Methods: Fifty-eight jSLE patients diagnosed according to the American College of Rheumatology classification criteria were included in this retrospective study. Disease activity of jSLE patients was measured by SLE Disease Activity Index-2K (SLEDAI-2K), organ damage was determined by the pediatric version of the systemic lupus international collaborating clinics/American College of Rheumatology damage index (pedSDI) at last visit. Results: Among 58 jSLE patients, the female patients accounted for 86.2% (n=50). Mean baseline SLEDAI-2K score was 21.47±8.96. The number of patients with at least one damage item of pedSDI was 24 (41.4%), in which 5 patients (8.6%) had AVN. There were no statistical differences between the groups except baseline complement 3 (C3) level was significantly lower in patients with AVN. Conclusion: Baseline low C3 level might be a predictor for AVN development in jSLE patients.There is unmet need for multicenter studies investigating possible risk factors of AVN in jSLE patients.

Association of Oxidative Stress with Disease Activity and Damage in Systemic Lupus Erythematosus: A Cross Sectional Study from a Tertiary Care Centre in Southern India.

To study oxidative stress in systemic lupus erythematosus (SLE) by estimating serum oxidised LDL (OxLDL), 8-hydroxy-2'-deoxyguanosine (8-OHdG), malondialdehyde (MDA), and total anti-oxidant status and to correlate with SLE disease activity and disease damage. Eighty SLE patients satisfying the Systemic Lupus International Collaborating Clinics/American College of Rheumatology (SLICC/ACR) 2012 criteria and 80 healthy controls were studied. Exclusion criteria were infections, renal insufficiency, other connective tissue diseases, drug-induced lupus, smoking, alcohol consumption. Disease activity was measured by SLE disease activity index-2K (SLEDAI), disease damage was quantified by SLICC-Damage Index (SDI). Sera was tested for OxLDL, 8-OHdG, and total antioxidant status (TAS) by double-antibody sandwich ELISA; MDA measured by Colorimetric assay. Oxidative stress markers were compared between group1- controls, group 2-mildly active SLE (SLEDAI ≤ 5), group 3- moderate to highly active SLE (SLEDAI ≥ 6). SLE patients had significantly higher MDA, 8-OHdG and lower TAS when compared to healthy controls, while OxLDL was similar in the three groups. MDA, 8-OHdG were significantly higher, TAS lower in group 3 compared to group 2. MDA had positive correlation with SLEDAI, TAS negatively correlated with SLEDAI. SLE with neuropsychiatric manifestations, vasculitis, anti-sdDNA antibodies had higher MDA, MDA/TAS ratio. SLE patients with thrombocytopenia, and vasculitis had higher OxLDL. Only OxLDL was significantly higher in those patients who have SDI > 1. SLE patients have increased oxidative stress measured by increases in MDA, 8-OHdG, and lower total antioxidant status that was associated with disease activity and some disease manifestations. However only OxLDL was associated with damage.

Novel Methods of Incorporating Time in Longitudinal Multivariate Analysis Reveals Hidden Associations With Disease Activity in Systemic Lupus Erythematosus.

Objective: Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease. SLE is characterized by high inter-patient variability, including fluctuations over time, a factor which most biomarker studies omit from consideration. We investigated relationships between disease activity and biomarker expression in SLE, using novel methods to control for time-dependent variability, in a proof-of-concept study to evaluate whether doing so revealed additional information.Methods: We measured 4 serum biomarkers (MIF, CCL2, CCL19, and CXCL10) and 13 routine clinical laboratory parameters, alongside disease activity measured by the SLE disease activity index-2k (SLEDAI-2k), collected longitudinally. We analyzed these data with unsupervised learning methods via ensemble clustering, incorporating temporal relationships using dynamic time warping for distance metric calculation.Results: Data from 843 visits in 110 patients (median age 47, 83% female) demonstrated highly heterogeneous time-dependent relationships between disease activity and biomarkers. Unbiased magnitude-based hierarchical clustering of biomarker expression levels isolated a patient subset (n = 9) with distinctively heterogeneous expression of the 17 biological parameters, and who had MIF, CCL2, CCL19, and CXCL10 levels that were higher and more strongly associated with disease activity, based on leave-one-out cross-validated regression analysis. In the remaining subgroup, a time-dependent regression model revealed significantly stronger predictive power of biomarkers for disease activity, compared to a time-agnostic regression model. Despite no significant difference in simple magnitude, using dynamic time warping analysis to align longitudinal profiles revealed a large subset (n = 69) with significantly stronger associations between biological parameters and disease activity. This subgroup had significantly lower flare rates, disease activity and damage scores, suggesting this clustering is clinically meaningful.Conclusions: These results suggest associations between biological parameters and disease activity in SLE exist in a multi-dimensional time-dependent pattern, with implications for the analysis of biomarkers in SLE often used to identify therapeutic targets. Novel methods to analyse high-dimensional data and control for time-dependent variability may have broad utility in the study complex relationships between clinical and biological parameters.

Correlation Between Cognitive Function with Disease Activity of Systemic Lupus Erythematosus Patients in Dr. Hasan Sadikin Hospital Bandung: An Analytical Cross-Sectional Study

Background: Cognitive dysfunction was found in 55-80% Neuropsychiatry Systemic Lupus Erythematosus (NPSLE) patients. Serious concern from clinicans was needed as its impact to patient’s quality of life. Disease activity is expected to be affecting patient’s cognitive function. Previous studies regarding correlation between disease activity and cognitive dysfunction showed various results. This study aimed to evaluate the correlation between disease activity and cognitive function in SLE patients.Methods: This study is an analytical cross-sectional study. Subjects were SLE patients at the rheumatology clinic of Dr. Hasan Sadikin Hospital Bandung during June-August 2017. Subject’s evaluations included disease activity assessment using SLE disease activity index-2K (SLEDAI-2K) and cognitive function assessment using MoCA-Ina test. Data were analyzed by using Spearman Rank correlation test. Results: Mean age of the subjects was 31 ± 8 years old, most of them were senior high school graduates (65.8 %) and median length of study was 12 years. Subject’s median duration of illness was 44 months. Their MoCA-Ina median score was 25, while SLEDAI-2K median score was 6. Cognitive dysfunctions were found in more than half of subjects (52.63%), which memory domain (78.95%) was most frequently impaired. Most of subjects were patients with active SLE (63.2%). Correlation test showed there was no correlation between SLEDAI-2K score and MoCA-Ina score (rs=0.023, p=0.445).Conclusion: There was no correlation between disease activity (SLEDAI-2K score) and cognitive function (MoCA-Ina score).&#x0D; Keywords: Cognitive dysfunction, MoCA-Ina, Systemic lupus erythematosus, SLEDAI-2K

Cardiac Manifestations in Systemic Lupus Erythematosus: Clinical Correlates of Subclinical Echocardiographic Features.

Objectives This study aims to correlate subclinical echocardiographic features with the clinical, laboratory, and therapeutic profiles of the patients to characterize risks for systemic lupus erythematosus (SLE) cardiac diseases. Methods The study included 59 SLE patients. Demographic data, disease characteristics, and current therapies were recorded, and the anthropometric measurements and routine laboratory tests were performed. The disease activity by the SLE Disease Activity Index-2K (SLEDAI2K) and the presence of metabolic syndrome (MetS) were assessed. Two-dimensional echocardiography was performed. Results The mean age of the patients was 31.3 ± 10.5 years, and the disease duration was 5.18 ± 4.1 years. 86.4% of the patients were females. Cardiac presentations by echocardiography were mainly mitral regurgitation (33.9%), tricuspid regurgitation (32.2%), mitral thickening (18.6%), aortic thickening (13.6%), pericardial effusion (13.6%), and pulmonary hypertension (8.5%) in order of frequency. The frequency of different echocardiographic findings with respect to other clinical phenotypes showed peaks with renal disease, MetS, and leukopenia. Components of MetS (triglycerides, high systolic blood pressure) and avascular necrosis were significant predictors for pericardial diseases (OR=1.011 CI 95% 1-1.022, p=0.046, OR=1.157 CI 95% 1.025-1.307, p=0.018, and OR=74.78 CI 95% 2.52-2215.76, p=0.013, respectively), and it is likely that hydroxychloroquine was protective against them. Age of the patients was a significant predictor for tricuspid regurgitation (OR=1.063 CI 95% 1.004-1.126, p=0.036). Mucosal ulcers were negative predictors for mitral thickening and regurgitation (OR=0.2 CI 95% 0.059-0.673, p=0.009). The use of corticosteroids appeared to protect against a number of valve lesions especially tricuspid regurgitation (OR=0.299 CI 95% 0.088-1.019, p=0.054). Conclusion This study highlighted different echocardiographic features and identified clinical predictors of different cardiac pathologies aiming to determine patients at risk and improve the prognosis of SLE cardiac diseases.

Predicting flares in patients with stable systemic lupus erythematosus

ObjectivesData on flares in Asian patients with systemic lupus erythematosus (SLE) are scarce. Here, we aim to identify the baseline predictors of flares in a cohort of Southeast Asian patients with SLE. MethodsConsecutive adult patients with prevalent SLE according to the 1997 ACR or 2012 SLICC criteria were enrolled and followed three-monthly. Clinical and laboratory data were collected at every visit using a standardised protocol. Flares were defined using the SELENA-SLEDAI Flare Index (SFI). Baseline predictors of flare in patients with stable disease (SLE Disease Activity Index-2K (SLEDAI-2K) of ≤ 4) were determined using Cox proportional hazards. ResultsOf the 210 patients recruited, 148 (70.5%) were Chinese. The median (IQR) SLEDAI-2K at entry was 2 (0–4) and the median (IQR) disease duration was 10 (4.4–16.4) years. At baseline, 152 (72.4%) patients had stable disease. After a median (IQR) follow-up of 31.5 (24.1–36.3) months, 109 (51.9%) flared. Stable patients who flared tended to be in the lowest tertile of age (HR 3.08, 95% CI 1.72–5.48, p < 0.01), had thrombocytopenia (HR 5.01, 95% CI 1.32–18.99, p = 0.02), hypocomplementemia (HR 3.35, 95% CI 1.54–7.30, p < 0.01) and had the highest baseline prednisolone doses (HR 2.39, 95% CI 1.28–4.46, p = 0.01). Conversely, patients in the lowest tertile of disease duration tended not to flare (HR 0.41, 95% CI 0.21–0.80, p = 0.01). ConclusionFlares are common in Asian SLE patients with initial stable disease. Close monitoring is needed for patients who are younger, with longer disease duration, thrombocytopenia, hypocomplementemia, or who required a higher baseline prednisolone dose.

Two-Parametric Immunological Score Development for Assessing Renal Involvement and Disease Activity in Systemic Lupus Erythematosus.

Objective Anti-double-stranded (ds) DNA and anti-C1q autoantibodies are useful tools in the assessment of disease activity and nephritis in systemic lupus erythematosus (SLE) patients. This study aimed to explore the utility of these antibodies along with anti-Ku antibodies in an oligoparametric model approach for the assessment of disease activity and lupus nephritis. Methods Samples from 261 well-characterized SLE patients were tested using chemiluminescent immunoassays (CIA) for anti-dsDNA and anti-Ku antibodies as well as by anti-C1q antibody ELISA (Inova Diagnostics, USA). Of these SLE patients, 26.4% had lupus nephritis (LN) at the time of blood draw or had a history of LN, and modified SLE disease activity index-2K (SLEDAI) scores were used to assess disease activity. Results All three antibodies demonstrated higher prevalence and higher antibody levels in active versus inactive SLE patients and in LN versus non-LN patients. When oligoparametric analysis was performed, the likelihood of LN and patients with active disease increased with dual and triple positivity. Conclusions Anti-dsDNA and anti-C1q antibodies are useful tools to identify disease activity and/or renal involvement in SLE patients. In addition, the combination of those antibodies in a two-parametric score might improve the clinical utility of those markers.

Sensitivity analyses of four systemic lupus erythematosus disease activity indices in predicting the treatment changes in consecutive visits: a longitudinal study.

This study was conducted to assess the ability of the British Isles Lupus Assessment Group-2004 (BILAG-2004), the SLE Disease Activity Index-2K (SLEDAI-2K), the European Consensus Lupus Activity Measurement (ECLAM), and the Revised Systemic Lupus Activity Measure (SLAM-R) to detect the need to treatment change in daily clinical practice. One hundred and two patients with SLE were enrolled and followed up for 2 to 8months and visited at least 3 times. Physician Global Assessment, BILAG-2004, SLEDAI-2K, SLAM-R, and ECLAM, were calculated in every visit. Treatment change, dependent variable, was categorized as decrease/no change vs. increase. The aforementioned indices, independent variables, were compared to learn their ability in predicting the treatment change. The probability of treatment change was measured by generalized linear-mixed effect model (GLMM) and generalized estimating equations (GEE). Adjusted odds ratios were calculated. Predictive power of indices was compared by area under the curve (AUC) in plots of sensitivity vs. 1-specificity and application of receiver operating characteristic curves (ROC). BILAG-2004 and SLEDAI-2K had substantial correlation with treatment change. Among different GLMM models, BILAG-2004 followed by SLEDAI-2K showed the highest associations with treatment change. Among various GEE models, similar findings were observed. Also, these 2 indices had the highest sensitivity (the largest AUC) towards treatment change; BILAG-2004 (AUC = 0.779, 95% CI = 0.710-0.848, p = 0.001) and SLEDAI-2K (AUC = 0.771, 95% CI = 0.698-0.843, p = 0.001). BILAG-2004 followed by SLEDAI-2K had the highest predictability of treatment change.

Prognostic factors of mortality in Iranian patients with systemic lupus erythematosus admitted to intensive care unit.

The aim was to determine the course, outcome, and determinants of mortality in patients with systemic lupus erythematosus (SLE) in intensive care unit (ICU). SLE patients admitted to ICU from 2004 to 2015 were recruited retrospectively. Demographic data, disease characteristics, causes of admission, baseline SLE disease activity index-2K (SLEDAI-2K) and Acute Physiologic and Chronic Health Evaluation II (APACHE) score, the outcome, and the causes of death were recorded. Predictors of mortality were compared between alive and dead patients by Cox regression analysis. Ninety-four patients with SLE were enrolled. Mean age at the time of ICU admission was 29.6years. Average scores of SLEDAI and APACHE II were 11.3 and 19.8, respectively. The most common causes of ICU admission were pneumonia, diffuse alveolar hemorrhage (DAH), and seizure. Forty-seven patients (50%) died in ICU. The principal causes of death were septic shock (25.5%), multi-organ failure (12.5%), DAH (10.6%), and pneumonia (10.6%). After multivariate analysis, high APACHE II, septic shock, and duration of mechanical ventilation were indicators of survival outcome. Mean (95% CI) survival days in ICU in patients with and without respiratory failure were 14.6 (10.4-18.9) and 28.7 (17.9-39.5) days, respectively (P=0.001). This figure for those with and without septic shock was 13.5 (4.9-11.1) and 22.3 (9.3-24.7) days, respectively (P=0.016). High APACHE II, septic shock, and duration of mechanical ventilation were the main predictors of death in patients with SLE in ICU. Multicenter studies are needed to draw a fine picture of SLE behavior in ICU.

Belimumab use, clinical outcomes and glucocorticoid reduction in patients with systemic lupus erythematosus receiving belimumab in clinical practice settings: results from the OBSErve Canada Study

To describe the characteristics of patients receiving belimumab, overall patterns of systemic lupus erythematosus (SLE) care, clinical outcomes, and changes in glucocorticoid dose following 6 months of therapy with belimumab, and healthcare resource utilization in belimumab users in Canadian clinical practice settings. Retrospective multicenter medical chart review study of adult patients with SLE who were prescribed belimumab as part of usual care and who received ≥8 infusions or 6 months of treatment. Primary endpoints included physician-determined overall clinical improvement from baseline, glucocorticoid use, and physician-determined SLE disease severity at Month 6. In total, 52 patients were included in the study. At belimumab initiation, 5.8/76.9/17.3% of patients had mild/moderate/severe SLE, respectively. Oral glucocorticoids were discontinued in 11.4% of patients and 59.1% received a lower dose at Month 6. At Month 6, 80.8/57.7/17.3% of patients had a physician-determined clinical improvement of ≥20/≥50/≥80%, respectively. Sixteen patients had a SLE Disease Activity Index-2K score at both baseline and Month 6, with a mean improvement of 2.6 ± 5.3 from 8.1 ± 3.2 at baseline. No formal disease assessment tool was utilized for 42.3% of study patients at baseline. This study provides the first real-world insights into belimumab use in Canada. It demonstrates significant reduction or discontinuation of glucocorticoid dose in 70.5% of patients and clinically significant improvement following 6 months’ belimumab therapy. The high number of patients with no formal disease activity assessments highlights a key care gap in SLE treatment in the real-world setting.