Lupus最新文献

IntroductionSystemic Lupus Erythematosus (SLE) is a chronic autoimmune disease that often manifests during productive years and frequently involves the central nervous system (CNS), including cognitive dysfunction, which occurs at twice the prevalence of the general population. While the Montreal Cognitive Assessment (MoCA) is effective for detecting mild cognitive impairment, no reliable biomarkers that indicate SLE patients at risk of cognitive dysfunction exist. Neuron-specific enolase (NSE), a specific marker of neuronal cell damage, has been shown in several studies to be highly expressed in cognitive dysfunction. This study serves as a preliminary investigation to examine the relationship between serum NSE levels and cognitive function in SLE patients.MethodsA cross-sectional study was conducted from January to August 2024 at Hasan Sadikin General Hospital, including SLE patients aged 18-55 years meeting the EULAR/ACR 2019 criteria. Exclusion criteria included pregnancy, neurological disorders (e.g., CNS infections, neurodegenerative diseases, stroke, epilepsy, head trauma), psychiatric conditions, substance abuse, systemic metabolic disorders, malignancy, other autoimmune diseases, or HIV. Cognitive function was assessed using MoCA-Indonesian version (MoCA-Ina) and serum NSE levels were measured. Associations between serum NSE and MoCA-Ina scores were analyzed using Spearman's correlation (p < .05).ResultsEighty-one participants (median age 32 years; 93.8% female) were included. Cognitive dysfunction (MoCA-Ina <26) was identified in 38.3%. Median serum NSE levels were higher in participants with cognitive dysfunction compared to those with normal cognition (14.0 ng/mL vs 12.7 ng/mL). Serum NSE levels showed a negative correlation with MoCA-Ina total scores (r = -0.225, p = .022) and executive function (r = -0.204, p = .034). Cognitive dysfunction was also associated with longer disease duration and a history of seizures.ConclusionThis study demonstrates a significant association between neuronal injury, as indicated by elevated serum neuron-specific enolase (NSE) levels, and cognitive impairment in SLE patients, with a particular impact on executive function. Longitudinal studies incorporating neuronal biomarkers are essential to provide deeper insights into the progression of cognitive dysfunction in SLE patients.

BackgroundSystemic Lupus Erythematosus (SLE) is a heterogeneous multisystem autoimmune disease, with variable severity, autoantibody profile, response to treatment, relapsing course and damage accrual. The age at disease onset may influence disease trajectory and prognosis, with remarkable differences of major organ involvement, disease activity, and prognosis. SLE clinical profile, activity indices, remission, and damage comparison were carried out in childhood-onset (cSLE), adult-onset (aSLE) and late-onset (lSLE) patients from a single-centre series.MethodsA cross-sectional analysis reviewing the clinical profile of SLE cases seen between 2012 and 2022 and classified in 3 age-groups according to disease onset: cSLE (<18 years), aSLE (18 to 49 years) and lSLE (>=50 years), was performed. Disease activity status was assessed by PGA (0-3), SLEDAI-2K, SLE-DAS, LLDAS and DORIS criteria, and damage using SDI at the same visit were compared in those 3 age-groups.ResultsFour hundred and fifteen patients were included in the analysis: 289 (68%) aSLE, 79 (19%) cSLE, and 47 (11.3%) lSLE. Most common clinical manifestations were articular (75.2%), hematological (70.1%), cutaneous (67.9%), photosensitivity (59.3%), and renal (41.7%). The clinical profile was similar among the subgroups of SLE patients, except for lSLE with lower frequency of nephritis and serositis, lower frequency of autoantibodies and hypocomplementenemia, lower SLEDAI-2K and SLE-DAS scores, and a higher frequency of LLDAS and DORIS remission; and a higher damage scores (SDI). Patients with cSLE had a significantly higher frequency of renal and neurological involvement, and a longer disease duration, but the disease activity, damage scores and remission rates were comparable to adult-onset group.ConclusionThe remarkable differences among the 3-age-groups were higher frequency of renal and neuropsychiatric domains in youngsters and disease activity scores compared to other age-groups. The lSLE group had less nephritis and serositis, lower disease activity and higher damage scores.

ObjectivesAvascular necrosis (AVN) is a common complication of Systemic Lupus Erythematosus (SLE) that causes significant morbidity for patients. This study aimed to determine the prevalence of symptomatic AVN in a large SLE cohort and to determine clinical and serological risk factors for symptomatic AVN overall and in early-onset and later-onset AVN subgroups.MethodsPatients with symptomatic AVN (n = 106) and those without AVN (n = 197) were identified in a cohort of 912 patients with SLE and followed up by a standard protocol in this retrospective case control study. Osteonecrosis was recorded when there was radiological evidence. Patients who developed AVN within the first year of SLE diagnosis were compared to those at a later stage as well as all AVN patients to 197 patients without AVN matched for disease duration. SLICC SLE damage index (SDI), and the disease activity (SLEDAI-2K) were determined at AVN diagnosis.ResultsThe prevalence of symptomatic AVN in our SLE cohort (n = 912) was 11.6% (n = 106). The mean age at SLE diagnosis of AVN patients were significantly lower than non-AVN patients (27.6 ± 10.6 vs 32.5 ± 12.6; p = .003). SLE-AVN patients had significantly higher alopecia, photosensitivity, oral ulcers, vasculitic skin rash, artritis, serositis, nephritis, pulmonary hypertension and neurologic involvement than non-AVN SLE patients (p < .05). Compared with the control group, SLE patients with AVN had significantly higher anti-cardiolipin IgG, lupus anticoagulant and anti-phospholipid antibody positivity of any kind (p = .020; 0.020; 0.018, respectively). In 106 patients, AVN was found in a total of 214 joints. Of the patients, 64.2 % (n = 68) had AVN in more than 1 joint. Patients who had AVN ≤1 year after SLE diagnosis had significantly higher mean SLEDAI-2K, higher mean daily steroid dose until diagnosis, and lower age (p = .041; 0.000 and 0.001, respectively). These patients also developed AVN at multiple joints significantly more than other patients (p = .047).ConclusionWe believe that controlling disease activity in the first year of SLE diagnosis and keeping daily steroid dose at minimum levels could be important in preventing early development of debilitating AVN in multiple joints, especially in younger SLE patients.

ObjectiveTo investigate whether obesity is a risk factor for chronic kidney disease G3 (CKD G3; eGFR <60 mL/min/1.73 m²) in lupus nephritis (LN).MethodsWe retrospectively reviewed 132 cases of biopsy-proven class III, IV or V incident LN for which quarterly data were available during a long follow-up period (median 140 months). Rates of complete renal remission, renal flare and CKD G3 were compared between obese (body mass index ≥30 kg/m²) and non-obese patients. Complete renal remission was defined as a urine protein to creatinine ratio (uPCR) < 0.5 g/g and a serum creatinine value <120 % of baseline. Renal flare was defined as the reappearance of an uPCR >1 g/g, leading to a repeat kidney biopsy and/or treatment change.ResultsBaseline characteristics of obese patients did not differ from non-obese patients. By contrast, time to CKD G3 and time to renal flare were statistically shorter in obese patients. Obesity significantly increased long-term risk for the progression of CKD [HR = 2.72 (CI95% 1.11-6.64), p = .028]. In a multivariate analysis, obesity was an independent risk factor for CKD G3 (p = .029).ConclusionA BMI ≥30 kg/m² is an independent poor prognostic factor for the progression of CKD in LN. More attention should therefore be paid to weight control in LN patients.

BackgroundPatients with Systemic Lupus Erythematosus (SLE) face a higher risk of cardiovascular morbidity, but data on Right Heart Failure (RHF) in-hospital outcomes in the context of SLE remain limited. Using a nationwide hospitalization database, we assess the impact of SLE on cardiovascular outcomes in cases of RHF.MethodsRHF cases were queried from the 2016-2019 National Inpatient Sample, comparing outcomes between those with vs. without SLE. Primary outcome included all-cause mortality while secondary outcomes included vasopressor use, acute kidney injury (AKI), mechanical ventilation use, hospital length of stay (LOS), and total hospitalization charges (THC). Multivariable and linear regression models adjusted for confounders including patient demographics and comorbidity burden.ResultsOf 5,569 RHF hospitalizations, 2% (111) involved SLE. SLE was associated with higher mortality (adjusted OR [aOR] 3.8, 95% CI 1.19-12.29), AKI (aOR 2.61, 95% CI 1.05-6.52), vasopressor use (aOR 8.11, 95% CI 2.20-29.8). No differences were observed regarding odds of mechanical ventilation use (aOR 1.39, 95% CI 0.35-5.5), mean LOS (7.3 vs 6.5 days, p = .436) or THC ($258,475 vs $86,910, p = .301) between both groups.ConclusionAmong RHF hospitalizations SLE is associated with higher mortality and non-fatal adverse outcomes. Further studies are necessary to confirm these findings and to clarify mechanisms aimed at improving outcomes for SLE patients hospitalized with RHF.

ObjectiveTo compare the efficacy of cyclophosphamide (IV CYC), mycophenolate mofetil (MMF), and tacrolimus (TAC), as induction treatment for lupus nephritis (LN).MethodsThis randomized, open-label, non-inferiority, active-controlled three-arm study included children and adult patients aged ≥10 years with clinical or biopsy-proven LN over a period of 1 year. Patients were randomized in a 1:1:1 ratio to receive IV CYC (0.5-0.75 g/m² monthly for 6 doses), MMF (2-3 g/day), or TAC (0.08-1 mg/kg/day). The primary outcome was the proportion of patients achieving renal response [complete (CR) or partial (PR)] at week 24 and secondary outcomes included the proportion of CR, PR, change in complements, anti-dsDNA antibody, and 24-hour urine protein levels, SLEDAI-2K and renal-SLEDAI scores from baseline to week 24. Serum CXCL10 was assessed at baseline and follow-up. Intention-to-treat analysis was performed.Results82 patients (94% females; median age 27.5 years) were randomized to receive IV CYC (28), MMF (27), and TAC (27). At 24 weeks, the renal response rates were 53.5%, 66.6%, and 62.9% in IV CYC, MMF, and, TAC groups, respectively. The lower limit of the confidence interval for the difference in renal response at 24 weeks between treatment groups was less than 20% (non-inferiority margin) (p = .58). Serum CXCL10 reduced significantly post-treatment in all three groups (p < .001). Three patients in the IV CYC group and one patient in TAC group died due to serious infections.ConclusionTAC was non-inferior compared to IV CYC and MMF for LN as induction therapy, with a comparable safety profile during the study period.

ObjectiveTo evaluate the concordance of four anti-dsDNA antibody detection methods-Crithidia luciliae indirect immunofluorescence test (CLIFT), enzyme-linked immunosorbent assay (ELISA), acridine ester direct chemiluminescence immunoassay (CLIA), and digital liquid chip method (DLCM)-and to assess their diagnostic efficacy in systemic lupus erythematosus (SLE) patients.MethodsA total of 285 serum samples were collected, including 170 SLE patients, 39 with non-SLE autoimmune diseases (AIDs), 28 with non-AIDs, and 48 undiagnosed cases. The concordance and diagnostic performance of anti-dsDNA antibody methods were analyzed.ResultsThe diagnostic performance showed that DLCM exhibited the highest sensitivity (86.87%), while CLIA demonstrated the highest specificity (94.03%). The area under the receiver operating characteristic (ROC) curve (AUC) was ranked as CLIFT < ELISA < CLIA < DLCM (AUC = 0.938). Anti-dsDNA antibodies detected by both CLIFT and DLCM correlated well with the SLE disease activity index (SLEDAI), while CLIFT and CLIA were significantly correlated with lupus nephritis. Utilizing ROC curve-derived cut-off values, the overall concordance of CLIFT and other methods ranged from 80.14% to 82.58% (kappa > 0.6, P < 0.001), and the concordance between quantitative methods ranged from 89.55% to 91.29% (kappa > 0.8, P < 0.001).ConclusionCLIFT, ELISA, CLIA, and DLCM all showed impressive diagnostic efficacy in detecting anti-dsDNA antibodies. CLIFT shows a strong correlation with SLE activity and lupus nephritis. DLCM, a relatively new method, also showed excellent performance and could be integrated into clinical laboratory workflows for anti-dsDNA antibody testing.

ObjectiveLupus nephritis (LN) is a serious manifestation of systemic lupus erythematosus (SLE). Existing data on LN in Saudi Arabia are primarily retrospective and derived from single centers. Therefore, this systematic review presents a unique and comprehensive analysis of the disease characteristics and outcomes of LN in Saudi Arabia.MethodA systematic search of MEDLINE, ScienceDirect, Embase, Cochrane, and PubMed databases was conducted up to April 2024. Eligible articles reporting on LN in Saudi Arabia were included, and disease characteristics and outcomes were analyzed.ResultsA total of 36 studies met the inclusion criteria. The prevalence of LN among SLE patients in Saudi Arabia ranged from 19% to 55%, with biopsy-proven LN reported in 11% to 42.7% of cases. Females accounted for 66% to 94.9% of patients, with a mean age at diagnosis between 23.8 and 39.7 years. Hypertension was present in 45.8% to 62.9% of patients, with nephrotic-range proteinuria in 20.7% to 58.5% and mean serum creatinine levels between 60 and 112.68 µmol/L. Class IV LN was the most common histopathological finding, occurring in up to 72% of cases. Complete remission was achieved in up to 81.5% of patients within 12 months, though lower remission rates were observed in those with proliferative LN. End-stage renal disease (ESRD) rates ranged from 5.5% to 30.7%, with proliferative LN, older age, and hypertension being significant predictors of progression to ESRD. Five-year survival rates ranged from 92% to 96%, while 10-year survival reached 95%. Infection and renal failure were the leading causes of mortality.ConclusionLN is highly prevalent in lupus Saudi patients, with Class IV LN being the most common and associated with poor outcomes. Although survival has improved, many patients still progress to ESRD. Prospective trials are needed to evaluate outcomes in the current era of biological therapies.

BackgroundSystemic Lupus Erythematosus (SLE), a complex autoimmune disorder characterized by altered immune regulation, particularly involving B cells, which exhibit increased survival and developmental dysregulation. This study aimed to investigate the expression of IRF4, IRF8, SP1, and PU.1 in B cells of SLE patients, as these factors are known to play critical roles in the development, function, and differentiation of B cells.MethodsB cells were isolated, cultured, and activated using anti-IgM. The mRNA expression of IRF4, IRF8, SP1, and PU.1 was assessed using reverse transcription polymerase chain reaction (RT-PCR) at baseline and after B cell activation. Correlations between transcription factor expression and clinical parameters were analyzed.ResultsUpon B cell activation, IRF4 expression increased significantly in SLE patients, unlike at baseline, where no changes were observed between groups. IRF8 expression was significantly raised in active SLE and increased upon activation. SP1 expression remained stable across all groups and conditions. PU.1 gene expression was higher in active SLE at baseline and increased further upon B cell activation. Positive correlations were found between IRF4 and IRF8, as well as between PU.1 and SP1. PU.1 expression correlated with SLE disease activity indices.ConclusionIRF4, IRF8, and PU.1 expression in B cells is altered in SLE. PU.1 shows a positive correlation with SLEDAI and anti-dsDNA titers. These findings highlight their potential roles in the pathogenesis of SLE in B cells.