Lupus最新文献

AimsShrinking lung syndrome (SLS) is a rare pulmonary manifestation of systemic lupus erythematosus (SLE) and affects approximately 0.5-1% of patients. This systematic review aimed to summarize the clinical features, imaging findings, therapeutic strategies, and outcomes of SLS in patients with SLE.MethodsThis systematic review was conducted in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. Studies published between 2015 and 2025 were identified using the terms "shrinking lung syndrome" AND "systemic lupus erythematosus." Clinical characteristics, imaging findings, pulmonary function tests, treatments, and outcomes were analyzed.ResultsA total of 52 cases were included, comprising 44 cases from the literature and 8 from a single-center case series. The mean age was 31.5 ± 7.5 years in our cohort and 38.6 years in the literature, with a marked female predominance. All patients had a prior diagnosis of SLE. Dyspnea was present in all the patients, followed by pleuritic chest pain and dry cough. The most common imaging finding was reduced lung volume with elevated diaphragmatic domes, which was observed in all the patients in our cohort and in 77.2% of the reported cases, followed by basal atelectasis. Diaphragmatic ultrasonography, performed in half of our patients, demonstrated reduced diaphragmatic excursion. Pulmonary function tests consistently revealed a restrictive ventilatory pattern with reduced diffusing capacity for carbon monoxide (DLCO). Immunosuppressive therapy as a corticosteroid-sparing strategy was used in all patients from our cohort and was the most frequently reported treatment in the literature, with rituximab being the most commonly used agent. Improvement in pulmonary function occurred in 62.5% of our patients and 50% of the reported patients, whereas stabilization was observed in approximately one-quarter of the patients. Two cases of clinical worsening, including one death, were reported.ConclusionSLS represents a potentially severe and underrecognized pulmonary complication of SLE. Despite the lack of standardized therapy, early diagnosis and timely initiation of immunosuppressive treatment beyond corticosteroids are critical for improving clinical outcomes, as evidenced by improvement in pulmonary function tests in approximately 52% of patients and the availability of effective steroid-sparing options. Prospective studies are needed to establish standardized treatment strategies.

ObjectivesPsychosis is a rare but severe neuropsychiatric manifestation of systemic lupus erythematosus (SLE). Its prevalence, clinical predictors, and immunopathogenesis remain incompletely understood. This study aimed to estimate the prevalence of psychosis in adult SLE patients and identify associated clinical and immunological risk factors.MethodsThis systematic review and meta-analysis was conducted following PRISMA guidelines. A comprehensive search of PubMed, Embase, and the Cochrane Library was conducted to identify all relevant studies with ≥10 adults through November 2024. Case-series, case reports, narrative reviews and conference abstracts were excluded. Quality assessment employed the Cochrane Risk of Bias Tool for randomized controlled trials and the Newcastle-Ottawa Scale for observational studies. Statistical analysis was performed using the random-effects model, with heterogeneity assessed via I² statistics.ResultsA total of 65 studies, comprising 31,495 SLE patients, were included. The pooled prevalence of psychosis in SLE patients was 4.5% (95% CI: 3.6%-5.5%) and 20.5% (95% CI: 10.0%-37.6%) among neuropsychiatric SLE (NPSLE) patients. Psychosis frequently occurred within 2 years of SLE onset and was strongly associated with higher SLE disease activity, positive anti-ribosomal P antibodies, antiphospholipid antibodies, and complement consumption. Delusions and hallucinations predominated among clinical presentations. Heterogeneity among studies was substantial.ConclusionObservational studies have reported that psychosis in SLE is strongly associated with increased disease activity and immune dysregulation. Anti-ribosomal P antibodies demonstrate a high negative predictive value, offering a valuable diagnostic adjunct. Recognition of psychosis may prompt closer clinical evaluation and, where appropriate, consideration of immunosuppressive treatment.

ObjectiveType I interferons (IFN) drive systemic lupus erythematosus (SLE) pathogenesis. Some patients develop neutralizing IFN autoantibodies (anti-IFN ab), which theoretically could modify disease activity. We aimed to determine the prevalence of anti-IFN ab in patients with SLE, identify the age and when during the disease course of anti-IFN ab emerge, and assess their association with organ damage.MethodsThis cross-sectional study included 173 SLE patients from the Lund Lupus Cohort. Samples taken at routine outpatient visits were analyzed for anti-IFN ab using ELISA, and positive samples were tested for IFN neutralizing capacity with a gene-reporter assay. Longitudinal samples were analyzed to determine the time-point and age of first positive sample. Demographic and clinical data were obtained from research registries.ResultsEighteen (10.4%) patients were positive for anti-IFN ab by ELISA. Among these, antibodies from nine patients (5.2%) displayed IFN neutralizing capacity. No statistically significant differences were detected between patients positive for neutralizing antibodies and antibody-negative patients with respect to demography, organ damage or ACR classification criteria. The group with neutralizing antibodies were slightly older (median age 59 vs 45 years, p = .14) and had a higher proportion of renal involvement (67% vs 33%, p = .088). Longitudinal analysis of samples from patients with neutralizing anti-IFN ab revealed two age-related patterns: late-onset (≥65 years, n = 4), including one patient positive at diagnosis at age 69, and early-onset (≤40 years, n = 5), with antibodies present at or soon after diagnosis in four cases. Organ damage did not differ between patients with or without neutralizing antibodies (p = .65). At the latest follow-up (2-38 years after anti-IFN ab detection), three of nine patients remained free of organ damage.ConclusionsApproximately 5% of SLE patients have neutralizing anti-IFN antibodies, which may present early in disease or develop later in life. While late-onset antibodies may reflect age-related changes in immune regulation and early-onset antibodies could potentially modulate IFN-driven mechanisms, our data do not support a protective effect against organ damage.

BackgroundSystemic lupus erythematosus (SLE) is a chronic autoimmune disease; the course of illness is interspersed with disease flares, infections, adverse drug events which may at times warrant intensive care admission. Reasons for admission and mortality predictors in intensive care units (ICUs) differ globally. This study offers a regional perspective by examining these factors in an Indian context.MethodsThis is a single centre ambispective observational study from a tertiary care hospital in South India. Patients with SLE admitted to ICU due to any cause between 2015 and 2021 were included. The primary objective was to describe reasons for admission to ICU among patients with SLE and secondary objectives were to determine short term mortality rate and factors predicting mortality. Patients' baseline disease characteristics, reason for current hospitalization and final outcome of mortality or discharge from hospital were studied from medical records. Descriptive statistics were used and logistic regression was used to predict in-hospital mortality with different variables.ResultsEighty-seven patients were included in the study. The mean age was 33.16 ± 12.6 years and 87% were female. The most common causes for admission were high disease activity with infection (48%) and high disease activity alone (39%). Mean APACHE II and SLEDAI-2K scores at admission were 17.32 ± 6.42 and 15.16 ± 8.3, respectively. The mean duration of ICU stay was 6.02 ± 6.58 days. The overall mortality rate was 29% with infection and disease activity being major contributors. In the multivariate model excluding cardiac arrest, APACHE II score (OR 1.56, 95% CI: 1.14-2.17, p = 0.010), RRT (OR 7.41, 95% CI: 2.86-20.00, p < 0.001), and lactate (OR 1.06, 95% CI: 1.01-1.10, p = 0.009) emerged as independent predictors of mortality.ConclusionThe leading cause of admission to ICU in our cohort was high disease activity with or without concomitant infection. The mortality rate in our study population was 29%, which is in line with the observed trend in the last 2 decades. APACHE II score at presentation, need for RRT, and baseline serum lactate emerged as independent predictors of ICU mortality.

BackgroundThe Intervention to Improve Quality of life for African American lupus patients (IQAN) Project is a three armed randomized, wait list-controlled trial focused on providing a variety of self-management tools to participants. We focus on African American individuals with lupus due to the increased morbidity and mortality in this population.PurposeTo examine whether a unique 'a-la-carte' self-management program improved quality of life, decreased depression, and reduced perceived and biological indicators of stress in African American lupus patients.MethodsIndividualized intervention plans (IIP's) offered 1-4 options, including a mail-delivered arthritis kit, message board, support group, and enrollment in a self-management program. A 'set menu' control condition included a standardized chronic disease self-management program only, and a control condition was usual care (UC). Validated measures of stress, depression, and quality of life were collected in all patients before and after intervention activities. To evaluate changes between baseline and post-intervention, compact scores were compared across groups, using two-sample t-tests.ResultsImprovements were observed in areas of stress management and pain management (p = 0.05). The frequency of managing pain by applying positive techniques increased in the intervention group (p = 0.08), but the other two groups did not display such improvements. An increasing trend persisted in the intervention group in the frequency of applying stress management techniques (p = 0.02) and decreasing trends in activity limitation were observed in both the intervention and set-menu control groups.ConclusionsBetter self-management outcomes were observed when participants were able to dictate the content or pace of the intervention program. This suggests that self-selection of program components has the potential to improve disparate trends in quality of life, disease activity and stress among African American lupus patients, which could impact future research and policy decisions.

ObjectiveBelimumab, a B-cell modulator, targets the central immunopathogenic pathway in systemic lupus erythematosus (SLE) by selectively inhibiting B lymphocyte stimulator (BLyS) and reducing the autoreactive B cells that drive disease activity. Its effectiveness in reducing disease activity and its steroid-sparing potential have been well-documented in both clinical trials and real-world studies. This study evaluated belimumab's long-term effectiveness in reducing oral glucocorticoid (OGC) use based on updated European Alliance of Associations for Rheumatology (EULAR) recommendations, and in attaining low disease activity and remission in adults with SLE.MethodsThis post hoc descriptive analysis (GSK Study 219649) utilized pooled data from individual OBSErve studies conducted in eight countries, collected at belimumab initiation and 6 months (all countries), and 6 to 24 months (USA and Argentina) post-initiation. Endpoints included percentages of patients achieving ≤5 mg/day OGC; maintaining 0 and ≤5 mg/day OGC beyond 6 months; attaining low disease activity (modified Lupus Low Disease Activity State [mLLDAS]: SLE Disease Activity Index [SLEDAI] score ≤4, OGC dose ≤7.5 mg/day) and remission (modified Definition Of Remission In SLE [mDORIS]: SLEDAI score = 0, OGC dose ≤5 mg/day) over time. The sample size in this study was fixed by available data from the OBSErve studies.ResultsData from 959 patients were included (mean [SD] age: 41.5 [12.4] years; 89.5% female; 52.2% from the USA). Of patients prescribed OGC at index, percentages receiving ≤5 mg/day increased from 16.1% at belimumab initiation to 51.6% at 6 months and 87.5% at 24 months; 8.2% discontinued OGC at 6 months and 44.4% at 24 months post-initiation. Of patients achieving 0 and ≤5 mg/day OGC at 6 months, 87.9% and 92.9% maintained this dose for 24 months. Percentage attaining mLLDAS/mDORIS increased from 0.4%/0.2% at belimumab initiation to 11.1%/7.4% at 6 months and 18.2%/12.9% at 24 months post-initiation.ConclusionsThese results from a real-world clinical setting suggest that belimumab treatment supports patients in achieving EULAR-recommended OGC taper goals, with sustained OGC dose reductions observed alongside an increased percentage of patients attaining low disease activity and remission as early as 6 months following belimumab initiation.

ObjectivesThe occurrence of a birth-cohort pattern underlying the time trends of any given disease is indicative of exposure to environmental risk factors during early life with long-lasting consequences that influence the disease occurrence during patients' subsequent lifetime. The present analysis serves to test whether the time trends of systemic lupus erythematous (SLE) in England & Wales and the United States are characterized by a similar birth-cohort patterns as other autoimmune diseases associated with Epstein-Barr virus (EBV).MethodsIn an observational study using the Vital Statistics of England & Wales and the United States from 1951 to 2022, the mortality trends of SLE were compared to those of Hodgkin lymphoma (HL), multiple sclerosis (MS), Crohn's disease (CD), and ulcerative colitis (UC).ResultsMortality from SLE rose among generations born during the 19^th century and decreased among generations born subsequently during the 20^th century. This birth-cohort pattern of SLE was matched by almost identical patterns underlying the occurrence of MS and CD, whereas mortality from HL and UC were similarly characterized by a birth-cohort patterns with a rise and fall in mortality that were shifted by 10-20 years towards earlier generations when compared to SLE, MS, and CD.ConclusionThe similarities in the birth-cohort patterns of SLE and other EBV-associated diagnoses suggest that they all share a common risk factor, such as EBV infection. The trends of SLE may have been shaped by underlying trends in the acquisition of EBV infection during adolescence or early adulthood.

BackgroundLupus choroidopathy was reported to be a marker of severe systemic erythematosus (SLE) activity and is frequently associated with nephropathy. However, it remains controversial whether choroidal thickness (CT) reflects glomerular vascular involvement or provides reliable indirect evidence of lupus nephritis (LN) activity. Therefore, the purpose of the present study was to assess the choroidal thickness in patients with active LN prior to the induction treatment and compare it with a healthy control group.MethodsThis case-control cross-sectional study evaluated 28 consecutive active LN patients before treatment initiation. All patients fulfilled the 2019 ACR/EULAR classification criteria for SLE, and LN was defined according to the American College of Rheumatology. Kidney biopsy-confirmed LN was present in 20 patients, with classification based on Renal Pathology Society/International Society of Nephrology standards. Health control group balanced by sex and age were included. CT was measured using the enhanced depth imaging protocol on spectral-domain optical coherence tomography.ResultsLN patients and controls had comparable median age (p = 0.445) and female predominance (p = 0.295). Renal parameters were characterized by median creatinine (0.80 ± 0.26 mg/dL) and elevated median protein/creatinine ratio (1.84 ± 1.70 g/g). Histological classes were predominantly proliferative [14/20 (70%)]. The mean central subfoveal CT was significantly lower in LN patients compared to the health control (297 ± 41.7 μm vs 329 ± 69.9 μm, p = 0.004).ConclusionThe observed thinning of central subfoveal CT in patients with active LN prior to treatment suggests that the choroid may serve as a subclinical target organ affected by systemic inflammation. Given its non-invasive accessibility, CT measurements may represent a promising tool for monitoring LN activity. Future longitudinal studies are warranted to determine its utility as a biomarker in the clinical management and follow-up of LN patients.

ObjectivePatients with systemic lupus erythematosus (SLE) often have concomitant fibromyalgia (FM) or similar symptoms including chronic pain, fatigue, or depression. This study explored whether Patient-Reported Outcomes Measurement Information System (PROMIS) measures provide richer information than 2016 American College of Rheumatology (ACR) FM criteria survey.MethodsPatients with SLE in our convenience cohort were categorized into groups: (1) concurrent FM chronic pain, (2) concurrent non-FM chronic pain, and (3) no chronic pain using 2016 ACR FM Survey. Based on PROs in the FM Survey, we captured comparable PROMIS measures (e.g., depression, fatigue). Associations by pain group were tested using Kruskal-Wallis rank sum test, Shapiro-Wilk normality test, chi-squared test, or Fisher's exact test. Violin plots explored differences across groups.ResultsThe cohort (n = 181) included 31 patients with FM pain, 23 with non-FM chronic pain, and 127 with no chronic pain. Median PROMIS symptom scores (fatigue, sleep disturbance, pain intensity and interference, depression) were highest and cognitive function lowest in the FM group, despite 13% being in remission. There were significant differences on 4 PROMIS measures (cognitive function, fatigue, pain intensity, pain interference) between FM pain and non-FM pain groups (p < .02), the former being worse. There were no significant differences in SLE Disease Activity Index (SLEDAI) score.ConclusionSLE patients with non-FM chronic pain have similar symptoms to FM compared with SLE patients without chronic pain; however, symptoms are not as severe as those meeting FM criteria. PROMIS measures may be used to classify severity more precisely for disease categorization and management.

BackgroundSystemic lupus erythematosus (SLE), an autoimmune disease, predominantly affects women and is associated with an increased risk of spontaneous abortion (SA). However, traditional analytical methods found a modest relationship between some factors and SLE-SA and were limited to a small sample size, frequently associated with poor predictive performance.ObjectivesThis study aimed to apply and evaluate an Extreme Gradient Boosting (XGBoost) model using routinely collected clinical data to identify patterns associated with spontaneous abortion in women with SLE and to identify the key variables associated with this outcome.MethodsThe study included adult SLE women from the Egyptian College of Rheumatology (ECR)-SLE cohort, a national multicenter study, which had available SA data. SA was defined as unexplained pregnancy loss up to 20 weeks of gestation. Patients' demographics, clinical manifestations, SLE disease activity index (SLEDAI), therapeutic and laboratory data were used as input variables for the logistic regression (LR) and XGBoost models. We evaluated the performance of both the XGBoost and LR models by calculating the area under the receiver operating characteristic curve (AUC) for each model, and then compared these AUC values to assess which model better distinguished between patients with and without SA. The importance and direction of each variable contributing to the risk of SA were evaluated using SHapley Additive exPlanation (SHAP).ResultsA total of 3296 SLE women (mean ± SD age: 32.5 ± 10.1 years; median disease duration: 48 months) were included. The mean SLEDAI score was 11.3 ± 9.5. About 13.9% of the patients included had at least one abortion. Optimized XGBoost performed better (AUC 0.99) compared with LR (AUC 0.78). Positive antiphospholipid antibodies, low complement 3, longer disease duration, hypertension and the presence of mucocutaneous ulcers, as well as anticoagulants and steroid use, were among the important factors associated with SA in SLE patients.ConclusionUsing information obtained in the clinical settings, the XGBoost identified variables associated with SA in women with SLE, including positive antiphospholipid antibodies, low complement 3 levels and longer disease duration. Further, longitudinal studies are necessary to evaluate the clinical utility of the proposed classification model.