Lupus最新文献

IntroductionLupus podocytopathy (LP) is an under-recognized pathological manifestation in patients with systemic lupus erythematosus (SLE). Despite being a distinct entity, current American College of Rheumatology (ACR)/European Alliance of Associations for Rheumatology (EULAR) guidelines do not include specific recommendations about LP, contributing to uncertainty regarding its diagnosis and treatment. This systematic review aims to synthesize the available literature on LP from case reports, case series and retrospective cohort studies to better characterize its clinical course, thereby informing clinical decision-making.Material and methodsA systematic search of EMBASE and MEDLINE was conducted to identify relevant studies. Eligible studies included case reports, case series, and cohort studies reporting SLE patients who had biopsy-proven lupus podocytopathy without features of class III, IV, or V lupus nephritis. Demographic characteristics, clinical presentations, relevant laboratory and pathology results, treatment and outcomes were studied.ResultsThis systematic review included 26 studies (18 case reports/small series and 8 cohorts), analyzing 19 individual cases and 240 cohort patients with LP. Most patients were young females, and LP was often part of the initial lupus manifestation with nephrotic-range proteinuria. Minimal change disease (MCD) was the predominant pathology. Patients with LP had an overall favorable outcome with treatment employing systemic steroid and steroid-sparing agents.ConclusionsLP is an uncommon but distinct manifestation of SLE with overall favorable outcome with treatment using systemic steroid and steroid-sparing agents.

Objective: We aimed to investigate in-situ expression of interleukin IL-17A, IL-21, IL-22, and IL-35 in lupus nephritis (LN).Methods: A cross-sectional study using immunohistochemistry (IHC) in renal biopsy to detect in-situ expression of interleukin IL-17 family and IL-35 from 20 Mexican patients with moderate to severe LN. Results: We found predominant expression of IL-17A and IL-21 in the glomerular region. IL-22 was detected in the tubulointerstitium and inflammatory aggregates, notwithstanding IL-21 and IL-35 in fibrotic and atrophic tubules. A positive correlation was found between IL-17A expression in the glomerulus and tubulointerstitium with creatinine, urea, and blood urea nitrogen levels, whereas IL-35 expression decreases in the presence of proteinuria. IL-21 was high in disease activity and severity versus IL-35 that decreased in systemic lupus erythematosus (SLE) disease activity.Conclusions: These findings provide evidence for the compartment-specific expression in LN of Th17-related cytokines and IL-35 in Mexican patients.

ObjectivesTo compare the efficacy of tacrolimus (TAC) plus glucocorticoids (GCs) with that of TAC plus mycophenolate mofetil (MMF) and GCs for the induction of remission in proliferative lupus nephritis (LN).MethodsThis multicentre cohort study, designed as a target trial emulation, included patients with biopsy-proven proliferative LN who received TAC-based induction therapy. Patients were classified into the TAC + GC and TAC + MMF + GC groups. The primary outcome was total renal response at 12 months, which was defined as a complete or partial renal response. To address baseline imbalances between the groups, inverse probability of treatment weighting (IPTW) was applied. Binary logistic regression was used to estimate the odds ratio (OR) for the renal response.ResultsIn total, 115 patients (48 with TAC + GC and 67 with TAC + MMF + GC) were included in the study. A 12-month total renal response was achieved in 16 (33.3%) patients in the TAC + GC group and 40 (59.7%) patients in the TAC + MMF + GC group (p = .009). After IPTW adjustment, the TAC + MMF + GC group showed significantly higher 12-month total renal response (IPTW-adjusted OR 2.84 [1.31-6.35]). Adverse drug reactions occurred in 7 patients in the TAC + GC group and 11 patients in the TAC + MMF + GC group.ConclusionsIn patients with proliferative LN, TAC + MMF + GC therapy was associated with a significantly higher 12-month renal response than TAC + GC. These findings support TAC + MMF + GC as the preferred TAC-based induction regimen for proliferative LN.

Childhood-onset systemic lupus erythematosus (cSLE) often presents with aggressive manifestations that may not respond to standard immunosuppressive therapy. We describe two paediatric cSLE cases with severe life-threatening complications-autoimmune hemolytic anemia in one patient and macrophage activation syndrome with acute respiratory distress syndrome in the other-successfully managed with Eculizumab as adjunctive treatment. Both patients demonstrated rapid stabilization after Eculizumab initiation and showed favorable outcomes at their latest follow-up. These cases highlight the potential role of complement inhibition in select cSLE complications and suggest that Eculizumab may serve as a rescue therapy beyond its established use in thrombotic microangiopathy. Further studies are needed to better define its optimal use and target patient population.

ObjectivesTo investigate the impact of time to diagnosis on damage accrual in thrombotic antiphospholipid syndrome (APS).MethodsRetrospective cohort study including 252 patients with thrombotic APS (Sydney criteria). Time to diagnosis was defined as the period from the first thrombosis to diagnosis (diagnostic delay ≥12 months). Recurrent events occurred during antithrombotic therapy. Damage was assessed using the damage index for APS (DIAPS). Risk factors for diagnosis and treatment delay (≥12 months) were assessed. Cox regression analysis was used to determine predictors of damage (DIAPS ≥1) and severe damage (DIAPS ≥ 3). Significance was set as α < 0.05.ResultsMost patients were female (76.7%) with primary APS (71.8%) and a median age at onset of 40.5 (28-51) years. The median time to diagnosis was 12 months (≥12 months, 53.6%). Damage and severe damage affected 75.4% and 21.0% of patients, respectively. Diagnosis delay was associated with higher cumulative thrombotic events, recurrent thrombosis and anticoagulation delay (all p < .001). Secondary APS (HR 2.65, 95% CI 1.52-4.62) and recurrent thrombosis (HR 2.66, 95% CI 1.52-4.66) predicted severe damage but not damage. Diagnosis delay did not predict damage but predicted severe damage (HR 2.99, 95% CI 1.25-7.16), even after multivariate analysis (aHR 3.18, 95% CI 1.24-8.12).ConclusionIn thrombotic APS, diagnosis delay is associated with increased number of thrombotic events and recurrent thrombosis, and is an independent predictor of severe organ damage.

IntroductionBlack patients with systemic lupus erythematosus (SLE) have lower medication adherence than White patients, contributing to worse health outcomes. However, racial differences in reasons for nonadherence and beliefs about medications are not well understood.MethodsWe conducted a cross-sectional analysis of Black and White patients with SLE who completed the Beliefs about Medicines Questionnaire and the SLE-specific Domains of Subjective Extent of Nonadherence survey. We compared scores by race and by adherence level within each racial group.ResultsAmong 123 patients (52% Black, 48% White), adherence was lower in Black patients (44% vs 64%, p = .02). Black patients reported greater concerns about SLE medications and medication overuse and harm than White patients. Nonadherent Black patients reported weaker beliefs in SLE medication necessity and greater concerns about medication overuse and harm than adherent Black patients. Reasons for nonadherence reported by Black patients but not White patients included feeling well (45%), concerns about future fertility (14%), and doubts about their doctors and medicines (8%).ConclusionNonadherence among Black patients was uniquely associated with stronger concerns about medication overuse and harm and weaker beliefs that SLE medicines were necessary, potentially reflecting medical mistrust that may drive skipping doses when feeling well or when concerns arise. These insights can help clinicians more astutely probe and address each patient's needs to enhance medication adherence and SLE management.

BackgroundLupusPRO is a disease-targeted, patient-reported outcome measure developed for assessing the quality of life in patients with systemic lupus erythematosus. Initially, the questionnaire was validated among U.S. patients of varied ethnic backgrounds and genders. This study aims to carry out a cross-cultural adaptation and validation of the Polish-translated version of LupusPRO.MethodWe administered the Polish version of LupusPRO along with the 36-Item Short Form Health Survey (SF-36) and EQ-5D-5L questionnaire, and the Polish modification of the Hospital Anxiety and Depression Scale (HADS-M). At the same time, we collected demographics and clinical characteristics. Disease activity, damage, and exacerbation were assessed using SELENA-SLEDAI, SLICC/ACR DI and LFA. Furthermore, we tested internal consistency reliability (measured with Cronbach's alpha), test-retest reliability (using r-Pearson correlation coefficient), convergent validity (against corresponding domains of SF-36) and criterion validity (against disease activity, damage and EQ-VAS), and known group validity.ResultsA total of 199 (91% females) patients with SLE with a mean age of 42.6 ± 12.62 years participated in the study. We observed that the mean SELENA-SLEDAI reached 5.3 (±5.9) points, while SLICC/ACR DI was at 1.3 (±2.0) points. The internal consistency reliability of LupusPRO domains ranged between 0.737 and 0.925 (except for Lupus Symptoms, Social Support, Coping, and Satisfaction with care). For all domains except Social Support, test-retest reliability exceeded 0.7. Convergent validity with corresponding domains of the SF-36 was good (r > 0.5). All health-related quality of life domains performed well against disease activity and damage measures, establishing its criterion validity. Confirmatory factor analysis showed a satisfactory fit. (± expression of range).ConclusionThe Polish version of LupusPRO has proved to have fair psychometric properties among Polish patients with SLE.

ObjectiveThis study explored whether modulating prefrontal alpha oscillations using transcranial alternating current stimulation (tACS) could alleviate depressive symptoms in individuals with systemic lupus erythematosus (SLE).MethodsOver the course of 5 days, three individuals underwent daily 40-min sessions of 2 mA bifrontal individual alpha frequency tACS (IAF-tACS) while watching a relaxing video. Resting-state 128-channel electroencephalography (EEG) was recorded on Day 1 and Day 5 before each tACS session. Self-reported assessments of depression, anxiety, insomnia, and fatigue were administered on Day 1, Day 5, and at the 2- and 4-week follow-ups.ResultsTwo out of three participants showed a reduction in left prefrontal alpha power after 5 days of IAF-tACS. Only those with this reduction reported short-term improvements in depression, insomnia, anxiety, and well-being.ConclusionThese preliminary results suggest that modulating prefrontal alpha oscillations through tACS may offer a potential approach for alleviating depressive symptoms in individuals with SLE.

ObjectiveTo compare and correlate cardiovascular risk (CVR) factors through ultrasound findings, clinical, and laboratory variables.Methods37 patients underwent ultrasound assessment, with CVR clinical scores applied: Framingham and QRISK-3 (Risk for Increased Cardiovascular Disease in SLE). The SLEDAI (Systemic Lupus Erythematosus Disease Activity Index) assessed systemic lupus erythematosus (SLE) activity.ResultsStatistically significant correlations were observed between the Framingham score, QRISK-3, and carotid artery diameter, with QIMT RF and expected values. Correlation coefficients ranged from 0.45 to 0.84, and p-values from 0.01 to <0.01. In linear regression analysis, variables influencing expected QIMT included QRISK-3, age, statin use, and diabetes mellitus, with p-values from 0.034 to <0.001. For QIMT RF, age and the Framingham score showed influence, with p-values from 0.028 to 0.004. SLEDAI did not significantly impact ultrasonographic parameters.ConclusionCarotid ultrasound was associated with in the subclinical detection of CVR in this cohort, providing complementary data to clinical evaluations.