Lupus最新文献

ObjectiveThe purpose of this systematic review study was to showcase diagnostic challenges posed by lupus mastitis (LM).MethodsHere we report a case of a 47-year- old Caucasian female with LM heralding systemic lupus erythematosus (SLE) complicated by macrophage activation syndrome (MAS), in the light of a systematic review of the literature on LM.ResultsIncluding our patient, we identified 32 case reports of patients with LM in the course of SLE. Only in 6 cases, including ours, LM preceded SLE diagnosis. Ours is the first ever published case report of LM heralding MAS.ConclusionLM in the course of SLE is rare and published data is very limited. Diagnosis should be made combining physical exam, laboratory tests, imaging and histology results, and include differentiation from other autoimmune, malignant and infectious causes. Based on the reviewed literature, it is advised to consider minimally invasive core biopsies in lupus patients over open biopsies as the associated trauma may exacerbate local inflammation. Conservative treatment with immunosupressive and anti-inflammatory drugs allows for control of breast symptoms in most cases.

BackgroundRecently, Cytotoxic T lymphocyte-associated antigen-4 (CTLA-4) gene has emerged as an attractive candidate gene implicated in susceptibility to autoimmune disease such as Juvenile-onset SLE (JSLE).ObjectiveTo investigate CTLA-4 exon 1 + 49A/G (rs231775) SNP as a genetic marker for susceptibility to JSLE and lupus nephritis in Egyptian children and adolescents.MethodsThis prospective case-control study included 260 patients diagnosed with Juvenile-onset SLE, and 260 healthy controls. We genotyped all participants for CTLA-4 (A/G) (rs231775) SNP located in exon 1 at position 49 by polymerase chain reaction.ResultsThe CTLA-4 exon 1 + 49G/G gene variant and G allele were significantly more represented in JSLE patients than healthy controls (27% vs 8%; ORs = 4.2; [95% CI: 2.4 - 7.3]; for the G/G genotype) and (47.5% vs 35%; ORs: 1.7; [95% CIs: 1.3 - 2.2]; for G allele); P < .01. The CTLA-4 G/G genotype and G allele were identified as possible risk factors for development of lupus nephritis (for G/G genotype; ORs: 5.09; [95% CIs: 1.7 - 13.9]; P = .0001, and for G-allele; ORs: 2.4; [95% CIs: 1.5 - 3.78]; P = .004).ConclusionThe CTLA-4 exon 1 + 49A/G polymorphism may confer susceptibility to Juvenile-onset SLE. Moreover, CTLA-4 G/G genotype and G allele at exon 1 + 49 may constitute independent risk factors for development of lupus nephritis in Egyptian children and adolescents.

BackgroundSystemic lupus erythematosus (SLE) is an autoimmune disorder marked by inflammation and immune dysregulation. Environmental factors like viral infections may contribute to disease onset. Cytomegalovirus (CMV), a beta-herpesvirus capable of immune evasion, has been proposed as a trigger in SLE pathogenesis, though studies show conflicting results.Materials and MethodsA literature search of PubMed, Scopus, Embase, Web of Science, and ScienceDirect identified eligible reports examining CMV prevalence and SLE association. Studies were selected using predefined criteria. Data were analyzed using Comprehensive Meta-Analysis (CMA) v4 software. Study quality was assessed using the Newcastle-Ottawa Scale (NOS). Heterogeneity and publication bias were evaluated using Tau square, Cochrane's Q, I² statistics, funnel plots, and Egger's regression test. Sensitivity analyses determined robustness.ResultsTwelve case-control studies comprising 1267 SLE patients and 1417 healthy controls were included. In the healthy control group, antibodies against CMV, specifically IgG (0.728) and IgA (0.641), were more prevalent than CMV DNA (0.095) and IgM (0.051). CMV DNA (OR: 9.727, 95% CI = 1.136 to 83.260, p = .038) and CMV IgM (OR: 2.630, 95% CI: 1.665 to 4.155, p = .000) were significantly more prevalent in SLE patients than in controls, suggesting a possible role for active or recent CMV infection in SLE development. Sensitivity analyses demonstrated overall consistency of findings; however, the association for CMV DNA showed variability across studies and should be interpreted with caution.ConclusionThis meta-analysis shows a link between CMV infection and SLE, suggesting CMV may be an environmental risk factor in SLE pathogenesis. Further research is needed to understand mechanisms and evaluate antiviral strategies.

BackgroundNeuropsychiatric systemic lupus erythematosus (NP-SLE) in children is uncommon but often severe, with a wide spectrum of neurologic presentations and sometimes atypical or initially normal neuroimaging. Among reported manifestations, acute cerebellitis is rare, and simultaneous thalamic involvement has scarcely been described. Differentiating inflammatory NP-SLE from mimics such as posterior reversible encephalopathy syndrome (PRES), infection, or ischemia is essential, since management strategies and prognosis differ substantially.CaseWe describe a 13-year-old female with newly diagnosed SLE who developed acute cerebellitis accompanied by bilateral medial thalamic signal abnormalities and non-convulsive status epilepticus after extensive infectious and cerebrospinal fluid evaluation. MRI demonstrated cerebellar cortical diffusion restriction and faint bilateral thalamic T2-FLAIR/DWI hyperintensity without corresponding ADC reduction, consistent with vasogenic edema, and without evidence of vascular occlusion. Management required high-dose corticosteroids, antiseizure therapy, plasmapheresis, cyclophosphamide, and rituximab. Her course was complicated by cytopenias, renal involvement, and encephalopathy, but she achieved gradual neurologic recovery with rehabilitation therapies and hydroxychloroquine maintenance.ConclusionsSimultaneous cerebellar and thalamic involvement expands the radiologic spectrum of pediatric NP-SLE and highlights thalamocerebellar network vulnerability underlying impaired consciousness. Recognition of this pattern supports early advanced MRI, continuous EEG monitoring, and prompt, multimodal immunotherapy to optimize outcomes.

ObjectiveThis study aimed to identify distinct cardiovascular risk phenotypes in systemic lupus erythematosus (SLE) using an unsupervised cluster analysis, and to compare the risk of incident cardiovascular events and subclinical atherosclerosis progression in the identified subgroups.MethodsConsecutive SLE patients who underwent a comprehensive cardiovascular risk assessment at the French National Referral Center for SLE between 2014 and 2024 were retrospectively included. An unsupervised analysis was performed using a hierarchical clustering algorithm on clinical, biological and imaging variables. Incident atherosclerotic cardiovascular disease (ASCVD) event rates, follow-up coronary artery calcium (CAC) scores and annualized CAC progression rates were compared across the identified clusters.ResultsA total of 226 patients were included (91% females, 45 ± 13 years). Three clusters were identified. Cluster 1 (N = 123) included young female patients with very few traditional cardiovascular risk factors, normal body-mass index (BMI) and low CAC scores. Cluster 2 (N = 78) included older female patients with a longstanding SLE course, high low-density lipoprotein levels and increased CAC scores. Cluster 3 (N = 25) included middle-aged male and female patients with frequent diabetes mellitus, increased BMI, triglycerides levels, epicardial adipose tissue volume and CAC scores. Overall, incident ASCVD events rates, follow-up CAC scores and annualized CAC progression rates significantly differed between the three clusters (C3>C2>C1, all P < .01). A post-clustering decision tree identified age, diabetes mellitus, and epicardial adipose tissue volume as key determinants of cluster membership.ConclusionSLE cardiovascular risk profiles encompass three subgroups with distinct cardiometabolic phenotypes and risk of incident cardiovascular events.

IntroductionSystemic Lupus Erythematosus (SLE) disproportionately affects African American women, who experience higher disease severity and face barriers to accessing supportive care. Peer mentoring offers a culturally tailored approach to improving chronic disease self-management and psychosocial well-being in underserved populations.AimsTo assess the impact of a peer-mentoring interventions on self-reported disease activity, symptom severity, and flare frequency among African American women with SLE.MethodsThis study is a sub-analysis of the Peer Approaches to Lupus Self-Management (PALS) randomized controlled trial. Adult African American female participants with SLE were assigned to an intervention, a social support control group, or served as peer mentors. The intervention group received 12 structured biweekly sessions over 24 weeks, delivered by trained peers using a culturally relevant curriculum. Outcomes were measured at baseline, 3, 6, and 12 months after the intervention using the Systemic Lupus Activity Questionnaire (SLAQ). Linear and cumulative logit mixed models were used to assess longitudinal changes, adjusting for sociodemographic covariates.ResultsThe intervention group reported sustained reductions in symptom severity over time, although not statistically significant. Notably, mentors demonstrated significant improvements in symptom severity at 3 months (mean difference: -2.53; 95% CI: -4.95, -0.11), suggesting reciprocal benefits of peer engagement. Employment and insurance status were consistently associated with lower symptom burden and disease activity.ConclusionThe results of our study support policy initiatives that invest in peer-based self-management interventions, expand insurance access, and address employment barriers shown to influence disease burden in individuals with SLE. Such efforts are critical to reducing health disparities and improving long-term disease outcomes.

ObjectiveMental health conditions, including anxiety, are common in childhood onset systemic lupus erythematosus (cSLE) and impact disease management and quality of life. This study evaluates the prevalence of anxiety symptoms and association of anxiety screening results with baseline and longitudinal demographic, clinical, treatment, and social features in cSLE.MethodsPatients ≥12 years of age diagnosed with cSLE were included. Demographic information, disease characteristics, disease activity, medications, provider-assessed medication adherence, and patient-reported outcomes of pain, physical function, anxiety, depression, suicidality and social determinants of health were extracted from the electronic health record. Anxiety symptoms, measured using the Generalized Anxiety Disorder (GAD) 7, were considered significant if score ≥10. The associations between GAD-7 scores and clinical, patient-reported, and social characteristics were evaluated longitudinally.ResultsAmong 76 cSLE patients with 196 visits, 47% and 21% had at least one GAD-7 of mild, and moderate or severe anxiety, respectively. Baseline clinical features including history of lupus nephritis and glucocorticoid dose were not associated with GAD-7 scores. The Deprivation and Community Index, which estimates socioeconomic disadvantage within geographic areas, did not vary by anxiety status. In multivariate longitudinal analysis, there was an association with depression and clinically significant GAD scores.ConclusionAnxiety symptoms were common in this cohort of cSLE, and anxiety and depression were significantly associated. In multivariate longitudinal analysis, there was no association between anxiety and disease activity, steroid use, pain, physical function, or social factors. Results support the need for routine anxiety screening in children and adolescents with lupus.

BackgroundSystemic lupus erythematosus (SLE) is an autoimmune disorder that affects various organs and follows a pattern of remission and relapse. Hydroxychloroquine (HCQ), an antimalarial drug, has recently become widely used in pregnant women with SLE due to its multi-level immune anti-inflammatory mechanisms and potential endothelial protective and thromboprophylaxis properties. While several studies have evaluated the impact of HCQ on SLE activity and the occurrence of neonatal lupus, its effects on improving early delivery, preeclampsia, and intrauterine growth restriction (IUGR) remain controversial.ObjectiveThis study aims to determine the effect of HCQ on feto-maternal outcomes among pregnant women with SLE.MethodologyIt is a retrospective cohort study over the past 25 years at Aga Khan Hospital, Karachi. The patients were divided into two groups. HCQ group had been taking HCQ throughout pregnancy. Non-HCQ group hadn't been using HCQ. All statistical analysis was performed using SPSS version 19.0. For all tests, p ≤ .05 was considered statistically significant.ResultsA total of 125 pregnant women with SLE were reviewed. The majority had conception in the remission period. There were 7 (20.6%) babies with fetal heart block in the non-HCQ group. The overall flare-up of disease was found in 68.8% (86/125), mostly in the third trimester. Positive anticardiolipin IgG antibodies were considerably higher in HCQ groups (47.25% vs 26.47%; p = .036).ConclusionOur study findings suggest that maintaining disease remission prior to conception and continuing HCQ therapy during pregnancy may be associated with improved maternal and fetal outcomes, though the observed association with fetal heart block warrants cautious interpretation due to small numbers.

ObjectiveCentral serous chorioretinopathy (CSC) complicating lupus nephritis (LN) may arise from two distinct pathways: uncontrolled systemic inflammation (activity-associated CSC) or as an iatrogenic complication of therapy (glucocorticoid-induced CSC). This study aims to propose a clinically actionable framework for differentiating these entities and guide trigger-specific treatment selection.MethodsWe present a novel case of glucocorticoid (GC)-induced CSC successfully treated with a GC-free belimumab-tacrolimus regimen. A case report integrated with a review of the literature (PubMed, Embase, Web of Science, until May 2024) was conducted to identify all reported cases of concurrent LN and CSC. Cases were stratified by presumed CSC trigger, and treatment outcomes were analyzed.ResultsFour cases, including our index case, were analyzed. Two cases of activity-associated CSC (no recent GC exposure) achieved dual remission with aggressive GC-based immunosuppression. Two cases of GC-induced CSC (onset post-GC initiation) only achieved CSC remission after implementing GC-sparing strategies (GC taper to ≤5 mg/d or cessation). A treatment-trigger mismatch (using high-dose GC for GC-induced CSC) was associated with worsened ophthalmological outcomes.ConclusionThese findings support a dichotomous pathogenesis model for CSC in LN. Correctly classifying CSC as activity-associated or glucocorticoid-induced is the critical first step in management. This distinction informs opposing therapeutic strategies: standard GC-based immunosuppression is appropriate for the former, while prompt initiation of GC-sparing therapy is imperative for the latter. This proposed framework offers a path to resolve the longstanding therapeutic paradox in this complex clinical scenario.

Background and ObjectivesPulmonary diseases (PD) are common in Systemic Lupus Erythematosus (SLE) and associated with increased mortality and decreased health-related quality of life, but no study has so far addressed PD in newly diagnosed patients with SLE. Our objectives were among newly diagnosed patients with SLE to investigate: Primarily, if PD and subtypes of PD are present, and secondarily characterise the patients by means of (i) lung physiology, (ii) radiology, (iii) thoracic ultrasound (TUS) and diaphragmatic ultrasound (DUS).MethodsPatients newly diagnosed with SLE from 1st July 2023 to 31st July 2024 at Odense University Hospital, Odense, Denmark, underwent a dedicated clinical evaluation for PD, including pulmonary function tests (PFT), chest high-resolution computed tomography or computed tomography scan alongside with TUS and DUS. Subsequently, PD were diagnosed, and subtype was categorised on a multidisciplinary discussion.ResultsTen participants were included in average 3 months after SLE diagnosis, and six out of ten had PD. PD included one case of shrinking lung syndrome (SLS) and two cases of interstitial lung disease. All participants exhibited at least one abnormal PFT measure, with some showing severely reduced pulmonary function. TUS was associated with diseases of the lung parenchyma and pleura and DUS with SLS.ConclusionWe found PD among newly diagnosed patients with SLE and in some cases associated with severely affected pulmonary function. TUS and DUS may contribute with information in diagnosing SLE related PD, but further studies are needed.