Lupus最新文献

BackgroundSystemic Lupus Erythematosus (SLE) exhibits marked sex-based difference. Understanding these sex-specific variations in clinical manifestations and autoantibody profiles, particularly within diverse ethnic cohorts like Chinese populations, is crucial for advancing personalized management strategies.MethodsWe retrospectively analyzed data from 1029 SLE patients (121 males, 908 females). Comprehensive immunological profiles, including complement C3, C4, CH50, antinuclear antibodies (ANA) titers and patterns, and antiphospholipid (aPL) antibodies, were assessed. Statistical analyses were performed to identify sex-specific prevalence and correlation patterns between autoantibodies and clinical indicators.ResultsDistinct autoantibody patterns emerged by sex. The speckled ANA pattern was more prevalent in males (84.1% vs 53.5%) and anti-Smith (Sm) antibodies were significantly higher in females (34.4% vs 19.8% in males; P = .001). No sex differences were found in ANA titers or aPL antibodies. Females had significantly lower complement levels and higher mean SLEDAI-2K scores (P < .001), while males showed more cardiovascular (P = .039) and pulmonary involvement (P = .026). Sex-specific correlations were evident: males displayed positive autoantibody-autoantibody correlations (e.g., anti-Sm vs anti-RNP), whereas females showed broader correlations, particularly between complement components and disease activity, serum creatinine, and anti-double-stranded DNA (dsDNA) antibodies, reflecting established markers of disease activity and renal involvement. Anti-Sm positivity linked to lower complement in both sexes. Notably, females with anti-Sm had increased anti-dsDNA positivity and elevated SLEDAI-2K, while males with anti-Sm showed higher 24-h urinary protein (P = .02), directly linking anti-Sm to renal involvement in males, a key aspect of male SLE severity.ConclusionsThis study highlights significant sex-based variations in autoantibody patterns and their clinical associations in a Chinese SLE cohort. The distinct prevalence of ANA patterns and anti-Sm antibodies, coupled with sex-specific immunological correlation networks, underscores the complex interplay of immune factors.

BackgroundCotrimoxazole (trimethoprim-sulfamethoxazole) is widely used for infection prophylaxis in immunocompromised patients, including those with lupus nephritis. Although generally well tolerated, rare cardiovascular adverse effects may occur and are not well recognized.Case presentationWe report the case of a 43-year-old man with newly diagnosed class IV lupus nephritis who developed a high-grade atrioventricular block (AVB) shortly after initiation of cotrimoxazole prophylaxis. The patient had no previous cardiac disease, normal renal function, and normal serum potassium levels at presentation. Cotrimoxazole was discontinued immediately, leading to complete recovery of atrioventricular conduction within 7 days. No recurrence occurred during follow-up.DiscussionWhile AVB has been associated with cotrimoxazole in the context of acute kidney injury or hyperkalemia, this case demonstrates a potential direct drug-induced nodal toxicity, as no metabolic or structural abnormalities were identified. The temporal relationship, absence of alternative explanations, and reversibility after drug withdrawal support a probable causal association between cotrimoxazole and AVB.ConclusionThis case highlights the importance of considering cotrimoxazole as a potential, reversible cause of atrioventricular conduction disturbances, even in patients with normal renal and electrolyte profiles. Clinicians should be aware of this possible complication and monitor electrocardiographic changes when prescribing cotrimoxazole, particularly in patients receiving immunosuppressive therapy.

ObjectiveTo evaluate whether hydroxychloroquine (HCQ) dose reduction (2-3 mg/kg/day) sustain lipoprotein levels achieved with higher doses of 2016-American Academy of Ophthalmology (2016-AAO) in stable lupus nephritis (LN) patients.MethodsForty-seven consecutive stable LN patients using HCQ 2016-AAO recommended dose for ≥6 months were enrolled and assigned to one of two groups: Reduced HCQ group (n = 21):LN patients who, upon inclusion, reduced 2016-AAO dose (2-3 mg/kg/day); and Maintenance HCQ group (n = 26):LN patients who continued on the standard 2016-AAO recommended HCQ dose (4.0-5.5 mg/kg actual body weight, maximum 400 mg/day) throughout 12-month study period. Blood HCQ levels, lipid profile and SLE parameters (including SLEDAI-2K) were assessed at baseline, 3 and 12-month.ResultsBaseline demographics, comorbidities and disease parameters were similar among groups (p > .05). Initial blood levels of Reduced HCQ group were 1219.4 (1041.7-1926.6)ng/mL and a progressive significant decrease were identified after 3 and 12 months [651.2 (538.4-832.6)vs.468.8 (228.4-925.6)ng/mL, p < .001]. Maintenance HCQ group had no changes in HCQ levels during the study [1179.7 (905.5-1607.3)vs.1026.2 (710.5-1345.8)vs.907.9 (663.9-1304.2)ng/mL, p = .158]. No changes were observed on the longitudinal total cholesterol levels of Reduced HCQ [166 (113-198)vs.153 (85-192)vs.150 (90-231)mg/dL, p = .964] and Maintenance HCQ [155 (114-244)vs.154 (122-210)vs.155 (112-213)mg/dL, p = .395]. LDL cholesterol levels of Reduced HCQ [91 (52-163)vs.83 (41-136vs.87 (47-165)mg/dL, p = .917] and Maintenance HCQ [79 (36-114)vs.76.5 (33-111)vs.75.5 (63-131)mg/dL, p = .412] remained similar. Other lipoprotein levels remained stable during 1 year of study.ConclusionThis is the first study to show that reducing HCQ to 2-3 mg/kg/day preserves lipid stability over 12 months in stable lupus nephritis patients.

BackgroundNeonatal lupus erythematosus (NLE) is a potentially life-threatening condition resulting from the transfer of autoantibodies from the mother to the fetus, with congenital heart block (CHB) its most serious complication. Despite the potential severity, therapeutic strategies remain limited. The objective was to systematically evaluate all registered clinical trials to date for the treatment or prevention of NLE and its cardiac manifestations.MethodsWe searched the Clinicaltrials.gov database on January 24, 2025, to identify all registered clinical trials for NLE treatments since 1994. This online registration of clinical trials is maintained by the NLM, a division of the National Institutes of Health (NIH), and mandates regular trial updates and timely results reporting. The registry provides specifics on a clinical trial, such as a national clinical trial (NCT) number, current recruitment status, study type, and other important data related to the trial. Principal investigators must submit results within 1 year of the trial's primary completion date.ResultsThree eligible trials for NLE were found on the NLM clinical trials database. One trial reported that hydroxychloroquine treatment reduced the recurrence rate of congenital heart block by more than half, from 18% to 7.4%. The remaining studies explored intravenous immunoglobulin and dexamethasone without posting results.ConclusionCongenital heart block from NLE was decreased in recurrence by treatment with hydroxychloroquine. However, the scarcity of research towards advancing care for these conditions highlights a major gap in this area.

Objectives(I) to study the association between antiphospholipid antibodies (aPL) and severe preeclampsia (PE) and (II) To assess the correlation between aPL and severe PE before and after 34 weeks of gestation.Study DesignThis prospective and interventional study included one hundred forty pregnant women separated in two groups based on their diagnostic status of PE.Control GroupForty normal pregnant women beyond 20 weeks of pregnancy. Tested for aPL: during they obstetric control and followed until birth. We only included volunteers who completed pregnancy without pathologies and with a normal full-term new-born baby.Study group (SG)One hundred women with diagnosis of severe PE. Tested for aPL: at time of severe PE onset and followed until birth. We stratified SG according to weeks of gestation at the time of diagnosis of severe PE (early and late PE), cut-off point of 34 weeks.We compare the frequency of aPL positivity between control versus SG.We've preformed two comparisons.a) Healthy Controls and patients with severe preeclampsia.b) In the study group comparison of early severe preeclampsia and late severe preeclampsia.ResultsWe compared the presence of aPL between Control 10.0% (4/40) and SG 34.0% (34/100). We found an association between severe PE and aPL positivity [OR = 4.63 (95% CI: 1.523 - 14.107), p = .005].We confirmed an association between early severe PE and the presence of aPL positivity [OR = 4384 (95% CI: 1.3615 - 14.1206), p = .01]. We also found an association between late severe PE and the presence of aPL positivity [OR = 5.0 (95% CI: 1.4901 - 16.777), p = .008].ConclusionThese results suggest that the presence of aPL is associated to severe PE. The positivity of aPL is associated to severity of PE, independently from gestational age.

BackgroundHealthcare disparities in SLE randomized clinical trials (RCTs) are well known, with minoritized persons being under-represented as trial participants despite facing more significant SLE morbidity and mortality. There is currently limited information as to how to best recruit a diverse group of participants to SLE clinical trials. Barriers to clinical trial participation can originate from either the patients or their clinicians. We therefore sought to investigate the effectiveness of educational curriculum in decreasing such barriers.MethodsWe utilized materials from the American College of Rheumatology, the Lupus Research Alliance, and the Lupus Foundation to develop educational curriculums for both clinicians and patients to see if we could improve their knowledge, attitudes, and skills regarding clinical trial participation.ResultsAttitudes towards clinical trials were highly favorable among patients and clinicians prior to the workshop and remained positive afterwards. The workshops increased the patients' comfort level regarding clinical trial participation, and increased clinician comfort with the clinical trial process and how to refer patients to clinical trials. Regarding knowledge about clinical trials, the results were mixed, with certain knowledge aspects such as the protections afforded to patients in RCTs and SLE disparities improving, but knowledge about the more logistical and regulatory aspects of RCTs not significantly improving. However, these improvements did not translate into increasing patient enrollment into RCTs or research registries.ConclusionAlthough our study did increase both patients' and clinicians' skill and comfort level regarding RCT participation, its small size did not allow us to observe concrete gains in RCT enrollment.

Background: Systemic lupus erythematosus (SLE) and antiphospholipid syndrome (APS) are complex autoimmune diseases with the potential to affect multiple organ systems and significantly impact quality of life. In the Middle East and North Africa (MENA), the burden of these conditions is amplified by a combination of genetic predisposition, environmental exposures, and socio-economic factors that shape both presentation and outcomes. High consanguinity rates, high ultraviolet exposure, infections, and lifestyle factors contribute to earlier onset and more aggressive disease, with lupus nephritis affecting up to 60% of patients. APS adds an additional layer of complexity through thrombotic events and pregnancy complications. Purpose: This review brings together current knowledge on the epidemiology, clinical patterns, and management challenges of SLE and APS across the region. Results: While individual country reports exist, the lack of large-scale registries limits our ability to fully define disease prevalence and outcomes. Delayed diagnosis, shortages of rheumatology specialists, and unequal access to advanced diagnostics and biologic therapies remain persistent barriers. In some low-resource or conflict-affected settings, even basic immunosuppressive medications are inconsistently available. The financial impact is substantial, driven by hospitalizations, medications, and loss of productivity, with many patients facing significant out-of-pocket costs. Recent advances, including biologics such as belimumab and anifrolumab, offer opportunities to improve outcomes but are not equitably accessible across the region. Conclusions: Moving forward, investment in healthcare infrastructure, training, and culturally appropriate patient education will be essential. Establishing regional registries, expanding research into genetic and environmental risk factors, and developing locally relevant management strategies are critical next steps. By addressing these gaps through coordinated action between policymakers, healthcare providers, researchers, and patient communities, it is possible to reduce the disparities in care and improve survival, function, and quality of life for people living with SLE and APS in the MENA region.

ObjectiveMembranous nephropathy can be categorised into idiopathic membranous nephropathy (iMN) and secondary membranous nephropathy (sMN). However, it should be noted that a subset of patients initially diagnosed with iMN may develop secondary etiologies during longitudinal follow-up, even in the absence of systemic manifestations at initial presentation.MethodsThis is a single-center retrospective analysis. From January 2017 to April 2024, patients who were diagnosed with iMN at the time of their initial renal biopsy, had no extrarenal lupus manifestations at that time, and subsequently developed lupus nephritis (LN) or systemic lupus erythematosus (SLE) during the follow-up period at our center were included. Patients with sMN and incomplete SLE at the time of the initial renal biopsy were excluded from the study.ResultsA total of six patients were included. Of these, five were female and one was male. The mean age of the patients was 30.00 ± 10.39 years. At the initial renal biopsy, the proteinuria level was 6.45 ± 1.83 g/L, the serum albumin level was 21.77 ± 3.69 g/L, and the estimated glomerular filtration rate was within the normal range. Notably, one patient exhibited a positive test result for the PLA2R antibody, with a titer of 135.5 RU/ml. Following the administration of immunotherapy, all six patients achieved remission. However, after a follow-up period of 7.00 ± 5.21 years, five of the patients experienced a recurrence. Repeat renal biopsies in four relapsed patients confirmed histopathological progression to LN (class Ⅲ+Ⅴ in two cases, class V in two cases). Two patients subsequently fulfilled the 2019 EULAR/ACR SLE classification criteria.ConclusionThe diagnosis of membranous nephropathy does not entirely exclude the possibility of secondary nephropathy, even in patients with a high PLA2R antibody titer. Consequently, close longitudinal follow-up for occult autoimmune disease remains a necessity in clinical practice.

PurposeThe Giscombé Superwoman Schema Questionnaire (G-SWS-Q) consists of five sub-domains. Systemic lupus erythematosus (SLE) is a chronic autoimmune disease that disproportionately impacts Black women and for which psychosocial mechanisms are of high relevance to well-being. The main objective of this study is to validate the G-SWS-Q factor structure among a sample of Black women living with SLE using confirmatory factor analysis (CFA).MethodsParticipants are part of Georgians Organized Against Lupus, a population-based SLE cohort in Atlanta. We measured SWS using the G-SWS-Q containing 35 items across five subdomains. We restricted to those identifying as Black women (n = 584). CFA was performed and model fit was assessed using standard fit indices and procedures for model re-specification in Mplus 8.1.ResultsThe mean age of respondents was 48 years (SD: 13.3)) and number of years of living with SLE was 16 (Median: 14.6, IQR: 7.9-21.22). All items were retained in the scale. After modifying the model by adding within latent factor error covariances, we obtained satisfactory model fit (RMSEA = 0.052; CFI = 0.90; SRMR = 0.063), which suggested that the factor structure is valid in this sample of Black women with SLE.ConclusionsOur findings confirm the factor structure suggested by G-SWS-Q for Black women living with SLE. The SWS has the potential to better understanding of psychosocial mechanisms underlying adverse health outcomes experienced by Black women by centering a gender- and race-specific orientation, with implications for engagement with health behaviors and disease management. In SLE, framing studies connecting chronic symptoms and their connection to quality of life with SWS may elucidate these mechanisms.