Lupus International Collaborating Clinics Damage Research Articles

Mortality in a cohort of Egyptian systemic lupus erythematosus patients: retrospective two-center study

BackgroundSystemic lupus erythematosus is a debilitating autoimmune disease with major contribution to the worldwide morbidity and mortality. This study aimed to investigate the causes of mortality in systemic lupus erythematosus (SLE) patients and the relation between clinical activity, disease-associated end-organ damage, laboratory markers and mortality.ResultsAmong the 771 patients who were successfully followed up, 34 patients (4.4%) died. The leading causes of death were infectious causes (35.29%), cardiopulmonary causes (26.48%), renal causes (14.7%), unknown causes (14.7%), neuropsychiatric causes (5.88%), and lastly gastrointestinal causes (2.94%). Subjects who died had lower complement 3 level, more anemia, lymphopenia, neutropenia, leukocytosis, thrombocytopenia, decreased glomerular filtration rate, higher incidence of infection, end-stage renal disease, and cardiopulmonary complications. Higher glucocorticoid dosage with more immunosuppressant (mofetil and cyclophosphamide) treatment was observed in patients who died. SLE disease Activity Index and Systemic Lupus International Collaborating Clinics damage index were both significantly higher in deceased persons. Multivariable hazards regression analysis revealed that lymphopenia (p = 0.017), decreased glomerular filtration rate < 50% (p = 0.002) with end-stage renal disease (p = 0.001), and high steroid daily use of > 40 mg (p = 0.016) were independent risk factors for the mortality of SLE patients.ConclusionInfections and cardiopulmonary complications are the leading causes of death in two centers caring for Egyptian SLE patients. Lymphopenia, end-stage renal failure, and high steroid daily use were associated with poor outcomes.

Patients with SLE have higher risk of cardiovascular events and mortality in comparison with controls with the same levels of traditional risk factors and intima-media measures, which is related to accumulated disease damage and antiphospholipid syndrome: a case–control study over 10 years

ObjectiveSLE is a strong risk factor for premature cardiovascular (CV) disease and mortality. We investigated which factors could explain poor prognosis in SLE compared with controls.MethodsPatients with SLE and population...

Apolipoprotein L1 risk genotypes in Ghanaian patients with systemic lupus erythematosus: a prospective cohort study

ObjectiveTwo apolipoprotein L1 (APOL1) risk variants (RV) are enriched in sub-Saharan African populations due to conferred resistance to Trypanosoma brucei. These variants associate with adverse renal outcomes by multiple causes...

Biomarkers of myocardial injury in lupus nephritis

Background Cardiovascular disease (CVD) is the main cause of death in patients with systemic lupus erythematosus (SLE) and lupus nephritis (LN). This study aimed to evaluate the prevalence of myocardial injury among patients with SLE without nephritis and patients with LN, determine whether serum high-sensitivity cardiac troponin T and creatine phosphokinase-MB (CKMB) might help to identify patients with LN at risk for CVD, and identify LN as a risk factor for myocardial injury. Patients and methods This study was conducted on 50 patients (30 patients with LN and 20 patients with SLE without nephritis). The SLE disease activity index (SLEDAI) and Systemic Lupus International Collaborating Clinics Damage Index (SLICC/DI) were assessed. Laboratory investigations, cardiac enzymes (Hs-TnT and CKMB), and transthoracic echocardiography were performed. Results The results demonstrated a higher prevalence of CVD in patients with LN. A total of 28 patients with LN (93.3%) vs 10 patients with SLE (50%) had detectable high-sensitivity troponin (Hs-TnT) levels, with a mean 29.77±18.22 vs 7.2±6.21 ng/l, respectively. The mean value of CKMB was higher in patients with LN than patients with SLE (23.9±18.6 vs 14.8±9.4 μg/l, respectively). Multilinear regression analysis for Hs-TnT revealed that low estimated glomerular filtration rate, high urinary albumin-to-creatinine ratio, low ejection fraction, and different ECHO diameters were the most statistically significant predictors of troponin elevation, with a P value less than 0.05. The strongest renal predictors of CKMB were estimated glomerular filtration rate and proteinuria, with a P value less than 0.05. Conclusion CVD is more in patients with LN than patients with SLE. Hs-TnT levels is a signature of subclinical cardiac disease, which could be used to identify at-risk individuals. CKMB and echocardiography lack adequate sensitivity for the diagnosis of myocardial injury in patients with LN.

Serum Levels of T Cell Immunoglobulin and Mucin-Domain Containing Molecule 3 in Patients with Systemic Lupus Erythematosus.

Objective: T cell immunoglobulin and mucin-domain-containing molecule 3 (TIM-3) is implicated in the development of various autoimmune diseases. We aimed to investigate the levels of soluble TIM-3 (sTIM-3) and their associations between clinical parameters in patients with systemic lupus erythematosus (SLE). Methods: Serum samples were collected from 65 patients with SLE and 35 age-matched healthy controls (HCs). The SLE Disease Activity Index 2000 (SLEDAI-2K) and the Systemic Lupus International Collaborating Clinics (SLICC) damage index (SDI) were used to assess SLE disease activity and SLE-related organ damage. British Isles Lupus Assessment Group (BILAG)-2004 index was also used to assess SLE disease activity. Soluble TIM-3 (sTIM-3) in sera from patients with SLE and HCs were evaluated by enzyme-linked immunosorbent assay (ELISA). The results were compared with the clinical parameters of SLE including SLE disease activity. Results: Serum sTIM-3 levels in patients with SLE (median 2123 pg/mL (interquartile range (IQR), 229–7235)) were significantly higher than those in HCs (1363 pg/mL; IQR, 1097–1673; p = 0.0015). Serum levels of sTIM-3 were correlated with disease activity of SLE using the SLEDAI-2K score (p < 0.001, r = 0.53). The serum sTIM-3 levels in SLE patients with active renal disease (BILAG renal index A-B) were significantly higher than those without the active renal disease (BILAG renal index C–E). However, no significant difference was observed in serum sTIM-3 levels between SLE patients with and without active involvement in other organs (BILAG index). Serum sTIM-3 levels were significantly elevated in SLE patients with organ damage (2710 pg/mL; IQR, 256–7235) compared to those without organ damage (1532 pg/mL; IQR, 228–5274), as assessed by the SDI (p = 0.0102). Conclusions: Circulating sTIM-3 levels are elevated in SLE patients, and serum sTIM-3 levels are associated with SLE disease activity and SLE-related organ damage. The data indicate a possible link between the TIM-3/Gal-9 pathway and SLE clinical phenotypes, and further investigation of the TIM-3 pathway in SLE pathophysiology is warranted.

Efficacy and safety of rituximab biosimilar in refractory lupus

AimsTo characterise patients with refractory SLE receiving rituximab biosimilar (CT-P10) and to explore short-term efficacy and safety associated with rituximab biosimilar use.MethodsWe retrospectively analysed data from the medical records of...

Validation of the Japanese version of the Lupus Damage Index Questionnaire in a large observational cohort: A two-year prospective study

Objectives The Lupus Damage Index Questionnaire (LDIQ) is a validated patient-reported outcome measure to assess accumulated damage in systemic lupus erythematosus (SLE). We aimed to translate it into Japanese and further investigate its validity and reliability. Methods The English version of the LDIQ was translated into Japanese and administered to Japanese patients with SLE (n = 259) at our university clinic. Physicians simultaneously completed the Systemic Lupus International Collaborating Clinics Damage Index (SDI) and SLE Disease Activity Index 2000 (SLEDAI-2K). Patients were prospectively followed for a repeat assessment the following year. Results The median LDIQ score was 2.0. The LDIQ demonstrated substantial correlation with the SDI but poor correlation with the SLEDAI-2K (Spearman’s ρ = 0.75 and −0.08, respectively). These results suggested its convergent and discriminant validity. The LDIQ demonstrated good test-retest reliability (intraclass correlation coefficient = 0.85). When the effect size and standardized response mean for the LDIQ were assessed only in patients who had a change ≥1 in the SDI, they demonstrated a small to moderate responsiveness: 0.43 and 0.59, respectively. Conclusion The Japanese version of the LDIQ had acceptable reliability and validity and its performance was comparable with the original version.

Superiority of cardiac magnetic resonance imaging over echocardiography in early detection of subclinical cardiac abnormalities in systemic lupus erythematosus patients

BackgroundCardiovascular disease (CVD) has become increasingly recognized as a cause of mortality, especially in patients with long-standing systemic lupus erythematosus (SLE). Aim of the workTo detect subclinical cardiac involvement and its relation to clinical characteristics, disease activity and damage. Patients and methodsTransthoracic echocardiography (TTE) was performed in 36 SLE patients. Cardiac magnetic resonance (CMR) sections were obtained. T1-weighted inversion recovery scout images were obtained after injection of gadolinium. ResultsThirty-six patients were included with a mean age of 32.4 ± 8.5 years; 35 females and 1 male; with disease duration of 7.9 ± 5 years. The frequent cardiac presentations on TTE were tricuspid regurgitation (TR) (41.6%), mitral regurgitation (MR) (36.1%), mitral thickening (25%), early diastolic mitral flow/mitral flow during atrial contraction (E/A) < 1 (19.4%). The most frequent cardiac presentations by CMR were MR (25%), pericarditis (25%), mitral thickening (13.9%), TR (13.9%), myocarditis (8.3). Neither SLE Disease Activity Index (SLEDAI) nor Systemic Lupus International Collaborating Clinics (SLICC) damage index, high- sensitivity-C reactive protein (hsCRP), C3 and C4 levels were significantly correlated with the ejection fraction (EF) by CMR. There was significant negative correlation between disease duration and EF by CMR (r = −0.36, p = 0.03). Using multiple regression, EF by CMR was strongly predicted by disease duration (p = 0.025). The analysis of EF and MR fraction by CMR and TTE showed acceptable moderate agreement. CMR and TTE showed 83.3% agreement in the detection of pericarditis. ConclusionCMR is superior to echocardiography in detection of subclinical abnormalities in SLE.

Sphingolipids and Diagnosis, Prognosis, and Organ Damage in Systemic Lupus Erythematosus.

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease that involves multiple organs and disproportionality affects females, especially African Americans from 15 to 44 years of age. SLE can lead to end organ damage including kidneys, lungs, cardiovascular and neuropsychiatric systems, with cardiovascular complications being the primary cause of death. Usually, SLE is diagnosed and its activity is assessed using the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI), Systemic Lupus International Collaborating Clinics Damage Index (SLICC/ACR), and British Isles Lupus Assessment Group (BILAG) Scales, which unfortunately often occurs after a certain degree of systemic involvements, disease activity or organ damage already exists. There is certainly a need for the identification of early biomarkers to diagnose and assess disease activity as well as to evaluate disease prognosis and response to treatment earlier in the course of the disease. Here we review advancements made in the area of sphingolipidomics as a diagnostic/prognostic tool for SLE and its co-morbidities. We also discuss recent reports on differential sphingolipid metabolism and blood sphingolipid profiles in SLE-prone animal models as well as in diverse cohorts of SLE patients. In addition, we address targeting sphingolipids and their metabolism as a method of treating SLE and some of its complications. Although such treatments have already shown promise in preventing organ-specific pathology caused by SLE, further investigational studies and clinical trials are warranted.

Trend of Survival of a Cohort of Chinese Patients With Systemic Lupus Erythematosus Over 25 Years.

Objectives: To revisit the trend of survival of systemic lupus erythematosus in a cohort of Chinese patients over 25 years.Methods: Patients who fulfilled the 1997 ACR criteria for SLE and were followed in our hospital since 1995 were included. Patients were stratified into two groups according to the year of diagnosis: (1) 1995–2004 and (2) 2005–2018. Survival of patients was studied by Kaplan–Meier analysis. Organ damage as assessed by the Systemic Lupus International Collaborating Clinics (SLICC) damage index (SDI) and causes of death in the first 10 years of SLE onset was compared between the two groups. Cox regression was used to study factors associated with survival.Results: A total of 1,098 SLE patients were registered in our database. After excluding 157 patients diagnosed outside the time period of 1995–2018, 941 patients were studied (92% women). All were ethnic Chinese. The mean age of SLE onset was 35.1 ± 14.4 years, and the mean duration of observation was 13.1 ± 6.6 years. Seventy-seven (8.2%) patients were lost to follow-up. Groups 1 and 2 consisted of 364 and 577 patients, respectively. The mean SDI score at 10 years of disease onset was significantly higher in group 1 than group 2 patients (1.01 ± 1.43 vs. 0.57 ± 0.94; p < 0.01), particularly in the neuropsychiatric, musculoskeletal, and gonadal domains. Within 10 years of SLE onset, 32 (8.8%) patients in group 1 and 25 (4.3%) patients in group 2 died (p = 0.005). The 5- and 10-year cumulative survival rates were 93.6 and 91.0% in group 1 and 96.5 and 94.2% in group 2 patients, respectively (log-rank test p = 0.048). Infection accounted for more than half of the deaths in both groups. More group 1 than group 2 patients died of vascular events, but the difference was not statistically significant. Cox regression showed that the age of SLE onset and damage score accrued at 10 years, but not the time period in which SLE was diagnosed, were significantly associated with mortality.Conclusions: The improvement in survival of our SLE patients is probably related to the accrual of less organ damage in the past 15 years.

Rubrics for mortality: a real-world observational long-term lupus nephritis cohort.

In this study, we aimed to evaluate long-term patient survival according to demographic data, clinical manifestations of systemic lupus erythematosus (SLE) and previous and current treatments, collected retrospectively. Patient selection required a minimum of four American College of Rheumatology revised criteria for SLE, biopsy-proven lupus nephritis (LN) available for reclassification according to the modified National Institutes of Health proposal for activity and chronicity indices and a minimum follow-up of at least three years since the last renal biopsy. Selection criteria were fulfilled in 25 patients followed for a median of 21 years. Based on the last renal biopsy, an equal number of patients were thus classified as class I/II and IV (n=8) and class III and V (n = 4). The mortality rate for LN was 14%. Having ever been diagnosed with glomerulonephritis (GN) type III or type IV but not class IV alone (p = 0.046), a higher histological chronicity index at the last renal biopsy (p = 0.022), not attaining renal remission one year after induction therapy (p = 0.004), end-stage renal disease on dialysis (p = 0.033) and the extra-renal Systemic Lupus International Collaborating Clinics Damage Index score (p = 0.017) were all significantly associated with mortality. Our results may provide important clues for strict observation protocols in particular categories of LN patients with long-standing disease.

Associations of serum soluble Fas and Fas ligand (FasL) with outcomes in systemic lupus erythematosus

ObjectiveFas/Fas ligand (FasL) and B cell-activating factor (BAFF) signalling have pivotal roles in SLE pathogenesis. We investigated the clinical associations of serum concentrations of soluble Fas (sFas) and soluble FasL...

Immunoglobulin G1 Antibodies Against Phosphorylcholine Are Associated With Protection in Systemic Lupus Erythematosus and Atherosclerosis: Potential Underlying Mechanisms.

ObjectiveImmunoglobulin M antibodies against phosphorylcholine (anti‐PCs) may be protective in atherosclerosis, cardiovascular disease (CVD), and systemic lupus erythematosus (SLE). We study immunoglobulin G1 (IgG1) and immunoglobulin G2 (IgG2) anti‐PCs, with a focus on atherosclerosis and SLE.MethodsWe determined anti‐PCs by using the enzyme‐linked immunosorbent assay in 116 patients with SLE and 110 age‐ and sex‐matched controls. For functional studies, we used three in‐house–generated, fully human monoclonal IgG1 anti‐PCs (A01, D05, and E01). Apoptosis was induced in Jurkat T cells and preincubated with A01, D05, E01, or IgG1 isotype control, and effects on efferocytosis by human macrophages were studied. Anti‐PC peptide/protein characterization was determined using a proteomics de novo sequencing approach.ResultsIgG1, but not IgG2, anti‐PC levels were higher among patients with SLE (P = 0.02). IgG1 anti‐PCs were negatively associated with Systemic Lupus International Collaborating Clinics (SLICC) damage index and Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) scores (odds ratio [OR]: 2.978 [confidence interval (CI): 0.876‐10.098] and OR: 5.108 [CI 1.3‐20.067], respectively) and negatively associated with CVD, atherosclerotic plaques, and echolucent plaques (potentially vulnerable plaques), but the association for the two former was not significant after controlling for confounders. D05 had a maximum effect on macrophage efferocytosis efficiency, followed by A01 and E01. The monoclonal antibodies showed differential binding specificity to PC and PC‐associated neoepitopes. A peptide analysis showed a difference in the complementarity‐determining region 3 of the three IgG1 anti‐PC clones that are crucial for recognition of PC on apoptotic cell surfaces and other neoepitopes.ConclusionIgG1 anti‐PCs are negatively associated with disease activity and disease damage in SLE, but the negative association with CVD is also dependent on confounding risk factors. One potential underlying mechanism could be increased clearance of dead cells.

Clinical and laboratory characteristics in juvenile-onset systemic lupus erythematosus across age groups.

BackgroundSystemic lupus erythematous (SLE) is a systemic autoimmune/inflammatory condition. Approximately 15–20% of patients develop symptoms before their 18th birthday and are diagnosed with juvenile-onset SLE (JSLE). Gender distribution, clinical presentation, disease courses and outcomes vary significantly between JSLE patients and individuals with adult-onset SLE. This study aimed to identify age-specific clinical and/or serological patterns in JSLE patients enrolled to the UK JSLE Cohort Study.MethodsPatient records were accessed and grouped based on age at disease-onset: pre-pubertal (≤7 years), peri-pubertal (8–13 years) and adolescent (14–18 years). The presence of American College of Rheumatology (ACR) classification criteria, laboratory results, disease activity [British Isles Lupus Assessment Group (BILAG) and Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2 K) scores] and damage [Systemic Lupus International Collaborating Clinics (SLICC) damage index] were evaluated at diagnosis and last follow up.ResultsA total of 418 JSLE patients were included in this study: 43 (10.3%) with pre-pubertal disease onset; 240 (57.4%) with peri-pubertal onset and 135 (32.3%) were diagnosed during adolescence. At diagnosis, adolescent JSLE patients presented with a higher number of ACR criteria when compared with pre-pubertal and peri-pubertal patients [pBILAG2004 scores: 9(4–20] vs. 7(3–13] vs. 7(3–14], respectively, p = 0.015] with increased activity in the following BILAG domains: mucocutaneous (p = 0.025), musculoskeletal (p = 0.029), renal (p = 0.027) and cardiorespiratory (p = 0.001). Furthermore, adolescent JSLE patients were more frequently ANA-positive (p = 0.034) and exhibited higher anti-dsDNA titres (p = 0.001). Pre-pubertal individuals less frequently presented with leukopenia (p = 0.002), thrombocytopenia (p = 0.004) or low complement (p = 0.002) when compared with other age groups. No differences were identified in disease activity (pBILAG2004 score), damage (SLICC damage index) and the number of ACR criteria fulfilled at last follow up.ConclusionsDisease presentations and laboratory findings vary significantly between age groups within a national cohort of JSLE patients. Patients diagnosed during adolescence exhibit greater disease activity and “classic” autoantibody, immune cell and complement patterns when compared with younger patients. This supports the hypothesis that pathomechanisms may vary between patient age groups.

Obesity increases the incidence of new-onset lupus nephritis and organ damage during follow-up in patients with systemic lupus erythematosus.

Objective This study explored the effects of obesity on clinical manifestations, disease activity and organ damage in Korean patients with systemic lupus erythematosus (SLE). Methods We assessed 393 SLE patients annually for three consecutive years based on demographic information, clinical manifestations, laboratory findings and Physician Global Assessment, Systemic Lupus Erythematosus Disease Activity Index (SLEDAI)-2000 and Systemic Lupus International Collaborating Clinics (SLICC) damage index (SDI) scores. Patients were grouped by body mass index (BMI): normal weight, BMI &lt;23 kg/m2; overweight, 23 kg/m2 ≤BMI &lt;25 kg/m2; obese, BMI ≥25 kg/m2. The impact of obesity on clinical outcomes was assessed using univariate and multivariate analyses. Results Of the 393 patients, 59 (15.0%) were obese at enrollment. They had more comorbidities compared with non-obese patients, including diabetes, hypertension, hyperlipidemia and pulmonary hypertension. Nephritis at enrollment and newly developed nephritis during follow-up were more common ( p = 0.002 and p = 0.002, respectively) and Physician Global Assessment and SDI scores were higher in these patients for three consecutive years ( p = 0.017 and p = 0.039, respectively). Multivariate analysis revealed that obesity was significantly associated with development of nephritis during follow-up (odds ratio = 26.636; 95% confidence interval, 11.370–62.399; p &lt; 0.001) and cumulative organ damage (odds ratio = 4.096; 95% confidence interval, 2.125–7.894, p &lt; 0.001). Conclusions The incidences of newly developed nephritis and cumulative organ damage were higher in obese SLE patients than in non-obese SLE patients.

Utility of the AVISE Connective Tissue Disease test in predicting lupus diagnosis and progression

BackgroundThe AVISE Connective Tissue Disease (CTD) test uses autoantibody, erythrocyte-bound C4d (EC4d) and B-cell-bound C4d (BC4d) levels to aid in diagnoses of SLE, other CTDs and fibromyalgia. We evaluated the...

Comparison of Disease Characteristics, Course and Outcome between Adults with Juvenile and Adult- onset Systemic Lupus Erythematosus

Abstract Aim of the Work This study was performed to evaluate disease characteristics, activity and damage in adult patients with either juvenile or adult onset SLE. Patients and Method One hundred and fifty consecutive patients with SLE fulfilling the SLICC criteria for the classification of SLE were included in the study. The patients were classified according to the age at disease onset, 100 Adult-onset (ASLE) and 50 juvenile onset (JSLE). Patients records were reviewed for clinical and laboratory evaluation. Disease activity was assessed using SLE Disease Activity Index (SLEDAI). Disease damage was assessed using Systemic Lupus International Collaborating Clinics (SLICC/ACR) damage index. Results The mean age at disease onset of JSLE was 14.120± 1.534 and of ASLE was 22.460± 2.732. The female to male ratio was 9:1 in JSLE compared to 13:1 in ASLE. JSLE had butterfly rash (66%), photosensitivity (30%), oral ulcers (34%), arthritis (44%) and neuropsychiatric manifestations (6%).While ASLE had butterfly rash (35%), photosensitivity (10%), oral ulcers (17%), arthritis (65%) and neuropsychiatric manifestations (13%). There was higher prevalence of anemia, thrombocytopenia, ESR, CRP, 24 h and creatinine in JSLE more than ASLE with statistically significant differences. The mean SLEDAI was significantly higher in JSLE (14.080 ± 4.780) compared to ASLE, (10.760 ± 2.738) (P &amp;lt; 0.001). Conclusion The age at onset of SLE influences its clinical manifestations and laboratory findings. JSLE patients have a more aggressive presentation and course than ASLE patients with a higher frequency of lupus nephritis, more cutaneous symptoms and hematological manifestations. JSLE patients have more severe disease activity with very high SLEDAI and SLICC damage scores. ASLE patients have more musculoskeletal manifestations.

The additive effects of hydroxychloroquine to maintenance therapy with standard of care in patients with systemic lupus erythematosus.

In this retrospective study, the effect of hydroxychloroquine (HCQ) added to maintenance therapy according to the standard of care (SoC) was evaluated for 1year in 101 patients with systemic lupus erythematosus (SLE). The primary endpoint was the SLE Disease Activity Index (SLEDAI). The secondary endpoints were the British Isles Lupus Assessment Group index, serum complement activity (CH50) levels, anti-double-stranded DNA (dsDNA) antibody titer, concomitant corticosteroid (CS) dose, and Systemic Lupus International Collaborating Clinics (SLICC) damage index. These variables were compared between the SoC+HCQ (n=42) and SoC (n=59) groups. The SLEDAI improved from 2 (0, 6) to 0 (0, 4) in the SoC+HCQ group (P = .038) but significantly deteriorated from 1 (0, 4) to 2 (0, 8) in the SoC group (P = .033). CH50, anti-dsDNA antibody titer, concomitant CS dose, and SLICC damage index did not significantly change. The increase in the SLEDAI and concomitant CS dose after 1year were all significantly greater in the SoC group, and the proportion of patients with SLEDAI flare was significantly lower in the SoC+HCQ group (SoC+HCQ: 4.76% vs SoC: 25.4%, P=.006). Univariate logistic regression analyses identified HCQ as a predictive factor for no SLEDAI flare (P=.003, odds ratio 6.81, 95% confidence interval 1.77-45.00). The use of HCQ effectively improved SLEDAI scores and was a predictive factor for the prevention of SLEDAI flare. Therefore, HCQ may be considered a potential mainstay of maintenance therapy.

Impact of fatigue on health-related quality of life and illness perception in a monocentric cohort of patients with systemic lupus erythematosus

BackgroundFatigue is a very common and debilitating symptom in patients with systemic lupus erythematosus (SLE), even among those with a mild or inactive disease. The objective of this study is...

Galectin-9 as a biomarker for disease activity in systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by elevated interferon (IFN) signature genes. Galectin-9 (Gal-9) is a β-galactoside-binding lectin that is reportedly useful as a biomarker for IFN gene signatures. In a cross-sectional study of Japanese patients with recent-onset SLE, we aimed to determine whether raised serum Gal-9 levels were associated with the disease activity or organ damage seen in SLE patients. The current study included 58 Japanese patients with SLE and 31 age-matched healthy individuals. Disease activity and organ damage were assessed using SLE Disease Activity 2000 (SLEDAI-2K) and Systemic Lupus International Collaborating Clinics (SLICC) damage index. Serum and cerebrospinal fluid (CSF) Gal-9 concentrations were quantified using ELISA. Correlation analyses between Gal-9 and clinical parameters including disease activity were performed. Serum levels of Gal-9 were significantly increased in patients with SLE compared with the control group (16.6 ng/ml, [interquartile range (IQR); 3.6-59.7] versus 4.74 ng/ml, [IQR; 3.0-9.5], p<0.0001). Gal-9 was significantly correlated with disease activity measures in the SLEDAI-2K. Serum Gal-9 levels were significantly greater in patients with SLE-related organ involvement (23.1 ng/ml, [IQR; 5.1-59.7] versus 12.5ng/ml, [IQR; 3.6-39.0], p = 0.013). Whereas there was no difference in serum levels of CXCL10 or M2BPGi between patients with and without SLE-related organ involvement. Serum levels of Gal-9 were significantly higher in SLE patients with active renal involvement determined by BILAG renal score (A-B) compared to those without active renal involvement (C-E). Whereas there was no significant difference in serum levels of Gal-9 between SLE patients with or without active other organ involvements (neurological or hematological) determined by BILAG score. SLE patients with detectable circulating IFN-α had raised serum Gal-9 levels. Levels of Gal-9 were significantly higher in the CSF from patients with recent-onset neuropsychiatric SLE (NPSLE) than in those from non-SLE controls (3.5 ng/ml, [IQR; 1.0-27.2] versus 1.2 ng/ml, [IQR; 0.9-2.1], p = 0.009). Gal-9 could be a serologic marker of disease activity and organ involvement in SLE patients. Future studies evaluating the role of Gal-9 in the SLE phenotype may provide insights into SLE pathogenesis.