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Background: Systemic lupus erythematosus (SLE), a rare multisystem disorder with a female preponderance, has a high cost for the care; however, there is no evidence relevant to the Indian setting. The primary objective of our study was to determine the financial burden of the index admission (IA) and subsequent costs during follow-up and ascertain the proportion with a catastrophic health expenditure (CHE). Methodology: This was an observational retrospective cohort study where inpatients of a general medicine ward were recruited from January 2019 to October 2020. Clinical details and costs were obtained from the hospital's electronic records and bills. Patients were telephonically interviewed for follow-up clinical details and costs incurred. A patient-family payer perspective was used. Linear regression analysis was used. Results: Of the 73 patients recruited during the study period, 96% were females and the majority (71%) were admitted through casualty, with 59% of patients having high disease activity (SLE Disease Activity Index >12). The hospital mortality was 9.6%. After a median follow-up of 12 months, there was good quality of life with no difference between the two severity groups. The total cost of the IA was 135,768 INR (94,053–223,954) and it was higher for the severe disease group (P = 0.038). The direct medical costs compromised 83% of admission costs. In the multivariate regression, the duration of hospital and intensive care unit stay were predictors of high cost. The median 6 months follow-up cost was 32,978 (14,240–80,940) and the total calculated annualized cost was 202,124 (136,188–331,508), which was not statistically different between the two groups. There was a CHE among 86% of patient-families. Conclusion: This study demonstrates that there is high morbidity and cost involved in the management of a flare of SLE. However, with appropriate care, there are reasonably good outcomes and quality of life beyond six months.

Introduction: In children with Systemic Lupus Erythematosus (SLE), autoantibodies are considered as biomarkers for specific organ involvement or tissue damage. Some autoantibodies are used for diagnosis, disease activity, and few for disease characterisation. By using cluster analysis, we identified antibody clustering in children with SLE with specific subsets of clinical manifestations at the time of diagnosis. Materials and Methods: All pediatric SLE (pSLE) who fulfilled 4/11 ACR criteria were included in this study. Their autoantibodies profiles were noted. We recruited 212 patients with newly diagnosed pSLE and cluster analysis was done. We identified 3 clusters which were used for analysis. Results: Cluster 1 had ANA and anti-dsDNA antibodies a low prevalence of all other antibodies. Children in cluster 2 had autoantibodies such as ANA, anti-dsDNA, anti-RNP, and anti-Sm. Cluster 3 was had autoantibodies such as dsDNA, ANA, anti-cardiolipin and anti-SSA antibodies. On analysis, there was statistically significant difference among the 3 clusters for hair loss (P = 0.006), oral ulcers (P = 0.024), arthritis (P = 0.025), neurological symptoms (P = 0.037), renal manifestations (P = 0.003), AIHA (P = 0.012). Conclusion: In pSLE, autoantibodies clusters with distinct clinical phenotypes. Hence, all autoantibodies should be done at time of diagnosis. It will help in predicting the clinical course of pSLE and also to identify patients at risk of developing major organ involvement later.

Background: Semaphorin-3A (Sema-3A) is an important immunoregulator protein; it has a role in the maintenance of self-tolerance, so it is thought to be involved in the pathogenesis of many autoimmune diseases such as systemic lupus erythematosus (SLE). The purpose of the study is to assess the possible role of serum Sema-3A level as a potential biomarker for disease activity in patients with SLE and its relation with lupus nephritis. Patients and Methods: We recruited fifty SLE patients and 25 healthy controls. According to the SLE disease activity index (SLEDAI), patients were divided into two groups; active SLE (n = 25) and inactive SLE (n = 25). Sema-3A level was assessed in the study groups using enzyme-linked immunosorbent assay. Laboratory work included antinuclear antibodies, anti-ds-DNA, C3, C4, C-reactive protein, and erythrocyte sedimentation rate (ESR). Results: Serum Sema-3A level was significantly lower among SLE patients compared to healthy controls (18.14 ± 5.77 vs. 65.72 ± 38.08, P < 0.001). Moreover, it was lower among active SLE group compared to inactive group (14.96 ± 4.27 vs. 21.32 ± 5.17, P < 0.001). Serum level of Sema-3A negatively correlated with SLEDAI (P ≤ 0.001) and ESR (P = 0.006) where it was correlated positively with C3 (P ≤ 0.001) and C4 (P = 0.017). Conclusion: SLE activity is associated with decreased serum level of Sema-3A, thus it is suggested that Sema-3A is a candidate to become a useful marker for SLE disease activity.

Background: Programmed cell death 1(PD-1)/programmed cell death ligand 1 (PD-L1) pathway is an immune checkpoint implicated in immune tolerance and involved in the pathogenesis of several autoimmune diseases. Systemic lupus erythematosus (SLE) is an autoimmune disease with multiple immune dysregulation. This study aimed to determine PD-1 and PD-L1 expressed levels on both CD3 T and CD19 B lymphocytes in SLE patients compared to healthy donors and their associations with the clinical data and disease activity of those patients. Patients and Methods: A total of 25 healthy donors and 80 SLE patients were involved in the study. PD-1 and PD-L1 expressed levels on each of CD3 T and CD19 B lymphocytes were determined in the peripheral blood (PB) using flow cytometry. Results: The expressed levels of PD-1 and PD-L1 on both CD3 T and CD19 B lymphocytes were significantly higher in PB of SLE group than that of controls (P = 0.01, P = 0.001, P = 0.009, and P = 0.001). Significant positive associations were found between PD-1 and PD-L1 expressions on both CD3 T and CD19 B lymphocytes with disease activity in SLE group (P < 0.05). Conclusion: PD-1 and its ligand PD-L1 could have a role as regulators for immune activation in patients with SLE.

Background: Diffuse alveolar hemorrhage (DAH) in systemic lupus erythematosus (SLE) is not very common but is associated with high mortality. No studies from India report on DAH in SLE. Materials and Methods: From the electronic database of SLE patients, data of those with unequivocal DAH between January 2008 and March 2017 were retrieved. Clinical, laboratory, treatment, and outcome details were noted. Univariate analysis and multivariate analysis were carried out with survival as an outcome measure. Results: Twenty-four (1.31%) of 1828 SLE patients had DAH. Ten patients had DAH at diagnosis of lupus. The median interquartile range age was 23 (19.7–30) years, the median duration of hospital stay was 14 (10–22) days, and the mean SLE disease activity index was 19.5 ± 8.29. The most common clinical symptom was dyspnea followed by hemoptysis. Nephritis was the most common extrapulmonary organ involvement in 18. Hypocomplementemia was noted in 19/20, double-stranded DNA positivity in 20 patients. The mean erythrocyte sedimentation rate (ESR) was 62.17 ± 39.92 mm 1st h. Concomitant infection was seen in eight patients and raised serum procalcitonin in four/15 patients. All patients received intravenous (IV) methylprednisolone pulses, cyclophosphamide in 20, IV immunoglobulin in 4, rituximab in 2, and plasmapheresis in 2. Twelve patients (50%) died. High ESR and mechanical ventilation were independent risk factors for mortality in patients with DAH. Conclusion: DAH in SLE is infrequent but is often associated with infection and high mortality. Mechanical ventilation and high ESR are associated with higher mortality.

Immunoglobulin G4-related disease (IgG4-RD) is a systemic autoimmune disorder with a variety of manifestations. Association with other systemic autoimmune diseases is uncommonly reported in the literature. Hence, we intended to understand better the uniqueness of the overlapping associations of IgG4RD with other systemic autoimmune diseases. Retrospective electronic medical records-based review of IgG4RD occurring as an overlap with other systemic autoimmune diseases seen in the rheumatology department was done. We present two cases of IgG4 disease overlapping with systemic rheumatic diseases with a brief review of the literature. The first case was an overlap IgG4RD with lupus and the second interesting case had IgG4RD overlapping with 2 autoimmune diseases (lupus and rheumatoid arthritis) in the same patient. Both patients were treated with high-dose steroids and mycophenolate mofetil with good response, however, the second patient needed to be additionally treated with rituximab for better control of arthritis. Although uncommon, here, we report the first case series of 2 patients with IgG4 disease overlapping with other systemic autoimmune diseases. In our case series, the presence of the overlap did not impact the immediate clinical outcome or treatment response of IgG4RD or the co-existent systemic autoimmune disease.

Background: Vaccine against COVID-19 is an effective preventive measure; however, systemic lupus erythematosus (SLE) patients were excluded from the vaccine trials, which leads to questions regarding vaccine safety and efficacy, giving rise to vaccine hesitancy. We aim to study the prevalence of vaccine hesitancy among SLE patients and study the factors affecting it. Methods: The study is a cross-sectional telephone-based survey done on SLE patients. The questionnaire included a series of 15 questions pertaining to their baseline characteristics, COVID-19 infection and vaccination details, and their perceptions regarding the COVID-19 vaccine. Nonvaccinated individuals were defined as “willing” and “hesitant” based on their intent to get vaccinated. Results: Among 418 (93% women) participants, about 8% had contracted COVID-19 infection in the past. Nearly half had been vaccinated against COVID-19, and 83% had experienced one or more side effects which were largely mild. Out of the 215 nonvaccinated participants, 84% were “willing” and 16% were “hesitant.” Among the reasons for hesitancy, hasty vaccine production, fear of SLE flares, general vaccine refusal and limited transport facilities were significant. On the whole, 65.1% had a positive attitude toward the vaccine. Conclusion: Despite a low-hesitancy rate, the number of SLE patients with a negative attitude toward the COVID-19 vaccine is reasonably high, with more than half of the patients yet to be vaccinated. This highlights that the mistrust and negative perceptions of the vaccine still persists. The role of health-care providers in vaccine acceptance in SLE patients is crucial.

Background: Virtual consultations have become the norm during the COVID-19 pandemic with disruption of regular outpatient services due to fear of infection and travel restrictions. Methodology: A telephonic survey was conducted among 54 patients with systemic lupus erythematosus (SLE) who sought virtual consultations to understand patients' perspective about these consultations. Results: 42/54 (77.78%) patients had inactive disease. Most patients (42/48 [87.5%]) did not visit the hospital for fear of COVID, whereas 58% of them also had travel restrictions. About one-fourth of individuals felt that consultation from home was comfortable (12/48 [25%]), cost-saving (14/48 [29.16%]) and they preferred virtual over physical consultation (14/48 [29.16%]). The satisfaction levels of patients during the process of booking appointment, the consultation process, and postconsultation queries also seem adequate. Conclusion: Virtual consultations in SLE are a feasible and conceivable option, especially for those with inactive disease on stable treatment.

Tjalma syndrome, also known as pseudo-pseudo Meigs' syndrome, is a rare manifestation of conditions in patients with systemic lupus erythematosus (SLE). The syndrome is characterized by the presence of ascites, pleural effusion, and an elevated cancer antigen-125 (CA-125) level. We present the case of a 27-year-old female patient admitted in 2021 without any comorbidities, who presented with unintentional weight loss, gastrointestinal upset, and ascites. Further evaluation showed elevated CA-125 levels and pleural effusions, with no atypical cells. The patient was initially treated with antiemetics, intravenous fluids, antibiotics, and total parental nutrition with no improvement in her symptoms or laboratory parameters. The results of an autoimmune workup met the criteria for the classification of SLE. After extensive investigation, she was diagnosed with Tjalma syndrome. She was subsequently treated with corticosteroids and hydroxychloroquine, resulting in the rapid resolution of the patient's nausea and emesis, and discharge from the hospital. Her ascites resolved over 4 weeks. Prednisolone was tapered down and azathioprine was added as a steroid-sparing agent. The patient eventually had complete remission of her symptoms, as well as remarkable improvements in her laboratory results. However, 8 months after her initial diagnosis, the patient showed signs of increasing SLE activity with lupus nephritis, anemia, and leukopenia, despite being compliant with her treatment regimen. The patient was initiated on oral prednisolone (1 mg/kg) and azathioprine was replaced with mycophenolate mofetil, which resulted in significant improvement of clinical and laboratory parameters within 3 weeks. Cases of Tjalma syndrome, and specifically, this presentation, are rarely reported in the literature. We present this condition to raise awareness about both the presenting symptoms and therapeutic options for Tjalma syndrome.

Objective: Fatigue is common in patients with systemic lupus erythematosus (SLE). Fatigue severity scale (FSS) is used to measure fatigue. This study was aimed to develop a culturally adapted Bangla version of the English FSS and to validate it. Methods: English version of FSS was translated into Bangla and adapted in the local sociocultural context, following the recommendations of Beaton et al. One hundred and twenty SLE patients were assessed in the Rheumatology Department of Bangabandhu Sheikh Mujib Medical University. Content validity of the adapted Bangla version was evaluated by item-level content validity index (I-CVI) and scale-level content validity index (S-CVI). Correlation between FSS and health assessment questionnaire-disability index (HAQ-DI) and 12 item short form health survey (SF-12) was measured for the assessment of convergent validity. Internal consistency and test–retest reliability were also assessed. Results: Our procedure had no problem with the content and language. I-CVI and S-CVI for each item was one. FSS showed strong positive correlation (>0.7) with HAQ-DI and strong negative correlation (>−0.7) with SF-12. The scale demonstrated excellent internal consistency (Cronbach's alpha = 0.93) and test–retest reliability (intraclass correlation coefficient = 0.93). Conclusions: The adapted Bangla version of FSS is a reliable and valid instrument and can be recommended for the self-assessment of fatigue in SLE patients.

Objective: The objective of this study is to identify factors associated with active tuberculosis (TB) in patients with systemic lupus erythematosus (SLE) and to study the impact of TB on SLE disease activity. Methodology: We screened case records of 643 individuals with SLE for the diagnosis of TB. SLE patients with TB (cases) (n = 29) were matched for age, sex, and disease duration in the ratio of 1:3 with controls (n = 94) (SLE without TB). Results: Pulmonary TB was seen in 41% (n = 12) and extrapulmonary TB in 59% (n = 17). Musculoskeletal TB (n = 6; 34.2%) was the most common extrapulmonary manifestation. TB was seen among those with lupus nephritis (55% vs. 38.4%) (P < 0.005) and those with autoimmune hemolytic anemia (48% vs. 38%). A higher median dose of daily glucocorticoids 11.5 (7.5–30) in 1 month preceding TB diagnosis was associated with a higher incidence of TB. Upon multivariable analysis, 1 month mean daily dose of glucocorticoids ≥ 7.5 mg (odds ratio [OR], 1.47; 95% confidence interval [CI], 1.07–2) was independently associated with TB. On a follow-up duration of 16 months, SLE flare was more among those with TB as compared to the controls (OR-2.49 95%; CI-1.39–4.48; P = 0.002). Conclusion: Extrapulmonary TB is the most common form in individuals with SLE. A higher steroid dose seems to predispose to TB infection. TB infection may increase the risk of SLE disease flare.

Aim: The present study was conducted to evaluate the incidence and characteristics of infections in patients of systemic lupus erythematosus (SLE) and determine the risk factors for infection. Materials and Methods: This is a prospective, observational study carried out at a single-center, tertiary care hospital. The study included all adult SLE patients diagnosed as per the revised American College of Rheumatology (ACR) criteria from January 2015 to October 2019. Episodes of infection and pathogens isolated were recorded. Results: A total of 100 patients with SLE were evaluated (94 females and 6 males). Forty-seven (47%) patients suffered from 54 episodes of infection. Urinary tract infections were most common (31.48%), followed by skin and mucous membrane (27.78%). Escherichia coli (E. coli) was the most common organism isolated in 9 (16.8%) cases. Tuberculosis was seen in 6 (11.1%) patients. Forty-three (79.6%) patients had a SLE disease activity index score of more than 5 at the time of infection. Significant risk factors for infection included use of prednisolone, prednisolone dose higher than 10 mg, use of cyclophosphamide within the preceding 3 months of infections, use of other immunosuppressive agents, and leukopenia. Renal lupus and presence of comorbidities did not have a significant association with occurrence of infections. The relative risk of infections in SLE patients was 4.7 compared to non SLE controls. Conclusion: Patients of SLE are at increased risk for various infections. Management of SLE needs vigilance for infection and judicious use of immunosuppressive drugs.

Objectives: This study aimed to determine whether mycophenolic acid area under the curve 0–12 h (MPA AUC0–12) concentrations correlate with the disease activity in children with lupus nephritis. Methodology: Twenty-five children who fulfilled the inclusion criteria were recruited. MPA levels were measured at specific intervals between 0 and 12 h of drug administration, including the baseline (trough) levels. Disease activity is determined by systemic lupus erythematosus disease activity index (SLEDAI) scoring along with laboratory parameters, and serological status was also compared with MPA AUC0-12 concentrations. Results: Out of 25 children, 20 (80%) were girls and 5 (20%) were boys. Mean MPA AUC0-12 concentrations was significantly lower in patients with active disease (SLEDAI score >6) (38.46 ± 14.3 μg.h/ml) than the patients with inactive disease (SLEDAI

Objective: The objective of the study was to evaluate the clinical, laboratory manifestations, and associations of macrophage activation syndrome (MAS) in a cohort of Pakistani patients with systemic lupus erythematous and to compare it with a well-characterized cohort of active systemic lupus erythematosus (SLE) without MAS. Materials and Methods: Patients with a diagnosis of SLE admitted from January 2017 to July 2019 were retrospectively reviewed. MAS ascertained by either clinical criteria or bone marrow biopsy were studied. Demographics, clinical, and laboratory parameters of MAS patients were evaluated and compared with a cohort of non-MAS active SLE. Results: A total of 305 patients of SLE were evaluated, out of these 18 patients had a diagnosis of MAS, making a MAS prevalence of 5.9%. Mean age of the patients was 27.25 ± 10.16 years. Female comprised 13 (72%) of MAS group. Fever was the most common clinical presentation (100%), followed by splenomegaly (38.9%) and lymphadenopathy (33.3%). At least 5 (27.8%0 patients had MAS as their initial manifestation. Anemia (88.9%), thrombocytopenia (88.9%), leukopenia (72%), and pancytopenia (55.6%) were prominent laboratory parameters. Mean serum ferritin of 1584 ng/ml and mean triglyceride of 270 mg/dl were reported. On comparing with non-MAS cohort; younger age, fever, lymphadenopathy, splenomegaly, mucocutaneous features, any type of cytopenia, high aspartate aminotransferase (AST), low albumin, and lactate dehydrogenase, all showed significant association with MAS (P < 0.05). Conclusion: Fever and associated cytopenias coupled with raised AST are strong predictors of MAS in patients with SLE.

Systemic lupus erythematosus (SLE) is a complex multi-organ autoimmune disease marked by the production of autoantibodies against nuclear structures, formation of immune complexes, and chronic inflammation triggered by their tissular deposition. SLE is characterized by alternating periods of relapse and remission and each flare has the potential to cause new organ damage related to either the disease process or the medication toxicity. Despite remarkable progress across its multiple domains, SLE is still an area with many unmet needs, calling for innovative and practical solutions. The efforts of the drug development programme in lupus have led to considerable growth in the last decade, owing to the approval of belimumab, anifrolumab, and voclosporin. The increasing understanding of the pathogenesis of the disease has enabled the exploration of novel therapeutic strategies. New discoveries in the intricate cytokine kaleidoscope of lupus have made the concept of targeted therapy an attractive and promising research focus. JAK inhibitors are oral targeted therapies approved for a wide variety of diseases across the Rheumatology, Gastroenterology, Dermatology, and Haematology fields. Multiple JAKis are currently being investigated in SLE. This paper aims to summarize existing data coming from both clinical trials and case reports regarding the use of JAK inhibitors in SLE., Competing Interests: Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Discoid lupus erythematosus (DLE) is a devastating autoimmune disease with few therapies available. For patients with little to no symptom improvement with initial treatment, the literature surrounding further treatment options and their efficacy remains limited. Here we report a 46-year-old patient with lupus and refractory DLE, who failed numerous medications since her initial diagnosis in 2014. She had a robust response to lenalidomide with further improvement after adding anifrolumab (ANI), in conjunction with the standard of care hydroxychloroquine. Furthermore, she was able to taper off steroids without interval flares. The patient has not experienced any major infections since the initiation of treatment. No previous case reports describing outcomes of lenalidomide and ANI have been reported, yet the combinational approach has potential. Future clinical trials are needed to investigate the safety of the combination of lenalidomide and ANI in lupus patients with refractory DLE., Competing Interests: Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Purpose: Systemic Lupus Erythematosus (SLE) significantly affects both the quality of life related to health and non-health aspects. This study aims to evaluate the psychometric properties of the Persian version of the Lupus&#95;PRO questionnaire, version 1.7. The questionnaire serves as a specific self-report outcome scale for assessing the treatment outcomes of Lupus disease., Methods: The Lupus&#95;PRO questionnaire, version 1.7, was translated into Persian using the standard forward-backward method. After being completed by 218 patients with Lupus, the psychometric properties of the instrument were examined. The Convergent and Discriminant Validity of the scale were assessed using Average Variance Extracted and Cross Loadings, respectively. The construct validity of the questionnaire was also evaluated through Confirmatory Factor Analysis (CFA). Cronbach's alpha was calculated to assess the reliability of the questionnaire dimensions., Findings: Out of the 218 patients with lupus who participated in the current study, 13 (6%) were male, and 205 (94%) were female. The mean (SD) age of participants was 40.29 (10.94) years. The Average Variance Extracted (AVE) for HRQOL and Non-HRQOL constructs, except for the Coping and Cognition dimension, was greater than 0.50, indicating satisfactory convergent validity. The interdimensional correlation coefficient (Discriminant Validity) for each dimension with other questionnaire dimensions was less than 0.10, indicating that the questionnaire has good convergent and discriminant validity. Additionally, the results of the Confirmatory Factor Analysis (CFA) indicated that the questionnaire was a suitable fit. In terms of reliability, Cronbach's alpha for the various questionnaire dimensions ranged from 0.51 to 0.91, indicating good internal consistency., Conclusion: The Persian version of the Lupus&#95;PRO questionnaire, version 1.7, demonstrates acceptable validity and reliability in the Iranian population. This instrument can effectively measure various aspects of the quality of life in patients with lupus., Competing Interests: Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Objective: Systemic lupus erythematosus (SLE) is a chronic autoimmune disease that affects multiple organs, notably the skin, joints, and kidneys. The primary goal in managing SLE is to enhance patients' quality of life (QoL). Illness perception can influence QoL in patients with chronic disease. We assessed illness perception in juvenile SLE (jSLE) patients and its effect on patient's and parental QoL., Method: Patients diagnosed with jSLE according to the SLICC 2012 criteria between January and November 2023 were included. Patients' illness perceptions were gaged using the brief illness perception questionnaire (B-IPQ), while patient's and parental QoL were evaluated using PedsQL and WHOQOL- BREF, respectively., Results: The study comprised 32 patients and 32 parents, predominantly female (78.1%). Musculoskeletal involvement was the most common (65.6%), followed by mucocutaneous (59.4%), renal, and hematological involvement (50% each). Neuropsychiatric involvement was absent. The median SLEDAI-2K score at the last outpatient clinic visit, which was documented in the patient's file was 2 (0-18) and was not correlated with the B-IPQ score (r = 0.121, p = .51). A significant negative correlation was found between B-IPQ and patient QoL, indicating poorer QoL in patients with negative illness perceptions (r = -0.576, p < .001). No correlation was observed between parental QoL and B-IPQ ( p => .05). Of note, 56.3% of patients had poor QoL, scoring below the PedsQL cut-off, while 43.8% of parents had poor QoL for general health, scoring below the WHOQOL-BREF cut-off for general health., Conclusion: Although disease perception did not correlate with disease activation in jSLE, it significantly impacted patient QoL. Enhancing patients' perceptions of jSLE may improve their overall QoL., Competing Interests: Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Introduction: Systemic Lupus Erythematosus (SLE) warrants an early diagnosis and prompt management. Delay in diagnosis can result in repeated flares, permanent damage, and even death. There is a large variability in the time taken to diagnose SLE across the world. We undertook this study to determine the time taken for diagnosis of SLE in India and to identify the factors associated., Methods: Patients with SLE diagnosed within the previous 1 year as per Systemic Lupus Erythematosus International Collaborating Clinics criteria (SLICC) 2012 criteria were included in a cross-sectional multicentre questionnaire-based survey. Demographic profile, self-reported socioeconomic status as per Kuppuswamy classification of socioeconomic status (version 2022) (SES), and several healthcare related parameters including referral pattern were recorded. Median time taken for diagnosis was used to demarcate early or late diagnosis and associated factors were explored., Results: We included 488 patients with SLE from 10 rheumatology centres. The median time to diagnosis was 6 months Interquartile Range (IQR 3,14.7) and within 3 months in about one third [150(30.7%)]. Very early diagnosis ( < 1 month) was established in 78(16.0%) patients. The mean SLE Disease Activity Index (SLEDAI) at diagnosis was 10.28 + 7.24. In univariate analysis, an older age, lower SES, non-southern state of residence and larger family size were significantly associated with late diagnosis. In the multivariate analysis, higher SES (AOR 0.95, 95% CI: 0.92-0.98), multiple organ system involvement at initial presentation (AOR1.75 95%CI: 1.08-2.84) and place of residence in south Indian states (AOR1.92 95%CI: 1.24-2.97) had lesser odds of being associated with late diagnosis. Distance from the closest medical centre/professional did not influence the time to diagnosis. Majority of patients had first consulted a medical graduate (42.5%) or postgraduate doctor (48.2%), and referral to rheumatologist was largely done by postgraduate (65%) doctors. More than half of our patients (61%) self-finance their treatment., Conclusion: Median time to diagnosis of SLE was 6 months, 1/3rd being diagnosed within 3 months and 78(16.0%) with 1 month of symptom onset. Delay in diagnosis was noted in those belonging to lower socioeconomic strata and those with single organ disease. Distance to the health care facility did not influence time to diagnosis., Competing Interests: Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Aim: To investigate the radial peripapillary capillary plexus vessel density (RPCP-VD) and peripapillary retinal nerve fiber layer thickness (pRNFLT) of systemic lupus erythematosus (SLE) and neuropsychiatric SLE patients (NPSLE) using disc optical coherence tomography angiography (OCTA) and investigate the association between these parameters and SLE disease activity index (SLEDAI-2K)., Methods: A total of 64 'right eyes (36 SLE patients, 28 healthy controls (HCs)) were included in this cross-sectional case-control study. Ten (27.7%) patients had neuropsychiatric involvement. RPCP-VD and pRNFLT of patients were evaluated in all peripapillary sectors. RPCP-VD and pRNFLT of NPSLE, non-NPSLE, and HCs were compared. The correlation between SLEDAI-2K and OCTA findings was evaluated., Results: SLE patients' RPCP-VDs were significantly lower compared with the HCs except for two sectors ( p < .005). There was not a significant difference in pRNFLT of SLE patients and HCs. There was not a correlation between SLEDAI-2K and RPCP-VD in any subsectors but there was a significantly negative correlation between pRNFLT in tempo-inferior and inferior-temporal sectors. When compared with non-NPSLE-patients, NPSLE patients had significantly lower inferior-hemi ( p = .001), inferior-nasal VDs ( p = .003), and peripapillary ( p = .012), superior-hemi ( p = .038), inferior-hemi ( p = .026), inferior-nasal ( p = .002) and inferior-temporal ( p = .012) pRNFLTs. A negative correlation was found between NPSLE and pRNFLT., Conclusion: SLE patients may have early subclinical vascular involvement leading to decreased RPCP-VD. A negative correlation between the SLEDAI-2K and pRNFLT in the temporal subsectors of all SLE patients may show an association between the disease activity and temporal pRNFL thinning. The presence of neuropsychiatric involvement may also be associated with decreased RPCP-VD and pRNFLT., Competing Interests: Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Background: Lupus nephritis (LN) is the most serious complication of systemic lupus erythematosus (SLE), and plasma exosomes may serve as a bridge. MicroRNAs (miRNAs) are abundant in exosomes, so this study aimed to explore the role of exosome-derived miRNA in the development of LN., Methods: The publicly available data containing plasma exosomal miRNAs in SLE patients and healthy controls were researched, and differential expression and functional enrichment analysis of exosomal miRNA was conducted. Then, plasma exosomes from SLE patients were extracted, and the accuracy of differential expression and functional enrichment analysis was preliminarily verified. PKH26 dye was used to label exosomes to detect whether exosomes can enter HK2 cells. Evaluation of plasma exosomes impact on cell viability was done by utilizing CCK-8 assay. Flow cytometry was used to measure cell apoptosis., Results: Plasma exosomes were successfully extracted and identified. Through differential expression analysis of the pulbilic data and subsequent qPCR validation, we observed that miR-20b-5p is overexpressed in plasma exosomes of SLE patients, whereas miR-181a-2-3p is downregulated. Then functional enrichment analysis revealed that these differential miRNAs primarily regulate processes such as apoptosis, autophagy, and inflammation. Then, flow cytometry analysis conducted after co-incubation of plasma exosomes and peripheral blood mononuclear cells confirmed that exosomes can indeed regulate apoptosis. And plasma exosomes can successfully enter HK2 cells without affecting cell activity. In addition, plasma exosomes promote HK2 cell apoptosis and autophagy. Overexpression of miR-181a-2-3p could inhibit HK2 cells apoptosis and upregulate the expression of bcl2, and beclin1. At the same time, a trend towards increased apoptosis rates was observed in HK2 overexpressed miR-20b-5p, although the difference did not reach statistical significance. And miR-20b-5p can enhance the expression of caspase3 and becin1 while suppressing the expression of bcl2 and LC3β., Conclusion: Our research indicates that the abundant presence of miR-20b-5p and the depletion of miR-181a-2-3p in plasma exosomes of SLE patients may mediate the promotion of apoptosis and autophagy in HK2 cells, thereby causing kidney damage and the development of LN., Competing Interests: Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Introduction: Systemic Lupus Erythematosus (SLE) can be diagnosed using the 2012 criteria of the Systemic Lupus International Collaborating Clinics (SLICC) and, more recently, the 2019 criteria of the European League Against Rheumatism/American College of Rheumatology (EULAR/ACR). Hematological involvement is scored differently by these classifications. Our objective was to compare both criteria in a cohort of children with autoimmune cytopenia (AIC)-associated SLE., Method: We included 79 patients with childhood-onset AIC as the first manifestations of SLE., Results: The median age at SLE diagnosis was 14.5 years (1.1-21.4 years). The SLICC criteria were fulfilled by 76/79 (96%) patients and the EULAR/ACR criteria by 72/79 (91%) patients during follow-up. The SLICC and EULAR/ACR criteria were discordant (not concomitantly fulfilled) in 25/79 (32%) patients. Non-hematological clinical manifestations were more frequently observed in SLE diagnosis when the criteria were concordant (30/54, 56%) than when they were not (5/25, 20%) ( p = 0.004). In 16/25 (64%) discordant patients, the SLICC criteria allowed earlier diagnosis of SLE. Finally, the attribution of a maximum weight of 6 to the hematological involvement of the EULAR/ACR criteria increased the sensitivity thereof from 63/79 (80%) to 76/79 (96%) in our population., Conclusion: The SLICC 2012 and EULAR/ACR 2019 criteria do not effectively diagnose SLE in children when AIC is the predominant feature. The SLICC criteria appear to be more effective in this population of SLE patients. An increase in the maximum weight of hematological involvement to 6 increases the sensitivity of the EULAR/ACR criteria for SLE diagnosis in children., Competing Interests: Declaration of conflicting interestsThe author(s) declare no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Background: Systemic lupus erythematosus (SLE) is a complex autoimmune disorder characterized by a malfunction of the body's immune defense system., Objective: The objective of the present investigation was to examine the expression and diagnostic significance of NRIR in SLE and to prove whether it is involved in the progression of SLE., Methods: The study involved 110 participants, including 55 healthy individuals and 55 SLE patients. The expression levels of NRIR, miR-31-5p, and ICAM-1 were measured using qRT-PCR. The ROC curve was performed to assess the diagnostic significance of NRIR in SLE patients. Pearson correlation analysis was utilized to explore the relationship between NRIR and other indicators. Cytokines including IL-4, IL-6, and IL-21, along with IgG levels, were assessed using ELISA. The interaction between NRIR and miR-31-5p was validated using a dual-luciferase reporter assay., Result: Upregulated expression of NRIR was observed in individuals with SLE, serving a diagnostic function for SLE. Additionally, abnormal expression of NRIR impacted the viability of CD4 + T cells within SLE patients. NRIR could negatively modulate the expression of miR-31-5p., Conclusion: LncRNA NRIR may be a potential biomarker for SLE and is likely involved in the progression of SLE., Competing Interests: Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Introduction: Systemic Lupus Erythematosus (SLE) is a chronic multi-systemic autoimmune disease that mainly affects young females. SLE's chronicity and high level of complications yield frequent clinic visitations & hospital admissions, increasing the necessity to investigate the healthcare system and improve patient satisfaction and quality of life., Objective: This study aimed to understand SLE patients' points of view on the healthcare system in Jordan, especially given the chronic nature of the disease. With a clearer understanding, improvements can be made to benefit both the patients and the healthcare system., Methods: A cross-sectional study of 79 patients following up at the University of Jordan Hospital, rheumatology clinics were interviewed over the phone., Result: The majority of patients were satisfied overall with the treatment services and medications as rated on a Likert scale of 1-5 (4.28 ± 1.01 and 4.19 ± 0.96, respectively) despite a quarter of patients complaining of adverse effects from the medications. The use of oral corticosteroids was significantly associated with a lower General Satisfaction Rate ( p = 0.050), while high income (1000 Jordanian Dinars and above) and fatigue contributed to a lower Medication Satisfaction Rate ( p = 0.016 and 0.000, respectively). A good physician-patient relationship was the most commonly cited reason for general satisfaction (73.4%) and was positively associated with the general ( p = 0.000) and medication satisfaction ( p = 0.004) rates., Conclusion: SLE patients perceived high satisfaction rates despite adverse effects and symptoms. These higher satisfaction rates were seen predominantly due to good physician-patient relationships., Competing Interests: Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Objective: Systemic Lupus Erythematosus (SLE) is a complex autoimmune disease predominantly affecting women. Despite advances in treatment, recent developments in single-cell RNA sequencing (scRNA-seq) and Mendelian randomization (MR) continue to facilitate the need for precision medicine., Methods: Data obtained from the GSE135779 dataset underwent quality control, normalization, and dimensionality reduction using Seurat and MonacoImmuneData. Marker genes identified subgroups for analysis with CellChat and ClusterProfilerR. MR analysis of these genes' eQTLs was performed to establish causal relationships with SLE using IEU Open GWAS project data., Results: Single-cell analysis revealed distinct cellular subtypes and highlighted increased monocyte levels in patients with SLE. MR analysis revealed 12 genes, particularly interferon induced protein with tetratricopeptide repeats 3 (IFIT3), causally related to SLE. Gene ontology and the Kyoto encyclopedia of genes and genomes analyses identified pathways significant to SLE pathogenesis. Visualization of these genes at the single-cell level revealed their role in disease progression. Cell communication differences between IFIT3-positive and -negative groups were also observed., Conclusion: This study demonstrates the potential of scRNA-seq and MR in identifying critical factors in SLE pathogenesis, thereby supporting the need for targeted therapies. Identifying IFIT3, among other genes, as central to SLE progression opens new avenues for precision medicine approaches in SLE management., Competing Interests: Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Objective: The aim of our study was to evaluate the renal response (RR) of three immunosuppressive protocols in the induction treatment of proliferative lupus nephritis (PLN) in a Tunisian population., Methods: We performed a retrospective prognostic cohort study in the Internal Medicine Department of the Habib Thameur University Hospital in Tunis from January 2000 to December 2023, and included kidney biopsy proven proliferative lupus nephritis patients. Three induction treatments were compared: High CYP regimen: glucocorticoids (GC) + IV cyclophosphamide (CYP) in monthly pulses of 0.7 g/m2 for 6 months; Low CYP regimen: GC + IV CYP in biweekly pulses of 500 mg for 3 months; and MMF regimen: GC + oral MMF 1.5 g twice daily for 6 months. The primary endpoint was the incidence of RR (complete and partial remission) at one year post-diagnosis. The additional outcomes were end-stage kidney disease (ESKD), severe adverse events (AEs) and death., Results: Our study included 78 PLN patients (High CYP: 17, Low CYP: 40, MMF: 21). The study found that 94.1% of patients receiving High CYP achieved the primary endpoint, RR, compared to 67.5% of those receiving Low CYP and 61.9% in the MMF group. For the additional outcomes, there were 3 cases of ESKD, all in the Low CYP group, 5 cases of death (4 in the Low CYP group and 1 in the MMF group), and 20 cases of severe AEs, all of which were severe infections (5 in the High CYP group, 12 in the Low CYP group, and 3 in the MMF group). Multivariate analysis showed that the High CYP regimen was more associated with RR than the MMF regimen, with an adjusted OR of 9.846 (95% CI: 1.087-98.210); p = 0.042. Multivariate analysis did not show statistically significant differences between the High CYP regimen and the Low CYP regimen in terms of RR., Conclusion: As an induction treatment for PLN, the High CYP regimen was strongly associated with a higher rate of RR than the MMF regimen. There were no statistically significant differences between the High CYP regimen and the Low CYP regimen in terms of RR., Competing Interests: Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Objective: This study aimed to evaluate the expression status of miR-132-3p in CD4 + T cells in patients with systemic lupus erythematosus (SLE) and explore its potential role in SLE development., Methods: The study included 60 patients with SLE and 30 healthy controls. miR-132-3p expression in CD4 + T cells was detected by real-time quantitative reverse transcription polymerase chain. Bioinformatics analyses were employed to predict target genes and explore the potential role of miR-132-3p. The associations between miR-132-3p levels and SLE Disease Activity Index (SLEDAI) score, as well as laboratory characteristics, were analyzed., Results: miR-132-3p levels in CD4 + T cells were significantly higher in patients with SLE compared with healthy controls. Bioinformatics analysis identified FOXO1 as a potential target gene of miR-132-3p, with a particular emphasis on the FOXO signaling pathway. miR-132-3p up-regulation in CD4 + T cells was associated with high SLEDAI score, high anti-double-stranded DNA levels, low C3 and C4 levels, positive anti-ribosomal P, and high 24-hour urinary protein levels in patients with SLE., Conclusions: miR-132-3p may contribute to CD4 + T cell dysregulation during SLE by targeting FOXO1 and could potentially be used to assess disease severity., Competing Interests: Declaration of conflicting interestsThe authors declared no potential conflicts of interest.

Objective: In Africa, the treatment outcomes of lupus nephritis (LN) are not well known. This is especially true in the current era where contemporary treatment options are more widely available. This retrospective study aimed to measure the outcomes of biopsy-proven LN treated at the Livingstone Tertiary Hospital (LTH) Renal Unit in Gqeberha (formerly Port Elizabeth), South Africa and to identify predictors of a poor outcome., Methods: A retrospective cohort study of 131 patients with biopsy-proven LN who had a kidney biopsy between 01 January 2012 to 31 December 2021 as identified from the biopsy register. A sub-analysis of 107 patients with proliferative and/or membranous LN was performed., Results: Mean age was 31.4 ± 12.7 years with a female predominance of 86.3%. At 6-month follow-up, 69.6% of patients had complete or partial response to treatment. This increased to 70.3% and 72.6% at 18 and 30 months, respectively. Twenty-seven patients were lost to follow-up, while 7 (5.3%) patients progressed to kidney failure (KF). There were 3 (2.3%) deaths. Predictors of poor response were an elevated baseline serum creatinine (OR = 2.53, 95% CI 0.99 - 6.52, p = .054), a decreased eGFR (OR = 2.92, 95% CI 0.94 - 9.09, p = .065) and an elevated blood pressure (OR = 6.06, 95% CI 1.11 - 33.33, p = .038) at the time of biopsy. Infections were the most common adverse event with 50 infections seen in 39 (29.8%) patients. Herpes viral infections were frequently noted ( n = 12) accounting for 24.0% of all documented infections., Conclusion: Response rates were similar in this cohort when compared to other contemporary studies. Predictors of poor response were an elevated baseline serum creatinine, a decreased eGFR and an elevated blood pressure at time of the biopsy. Infections were the most common occurring adverse event, although the mortality rate remained low at 2.3%., Competing Interests: Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Objective: This study aims to explore possible susceptibility genes and clinical features for systemic lupus erythematosus (SLE) patients in a Chinese population., Methods: Expanding on the results of a prior single-center observational study involving 60 systemic lupus erythematosus patients, a subsequent single-center prospective observational study was conducted on SLE patients undergoing treatment at Nanfang Hospital Affiliated to Southern Medical University from 2021 to 2023. The identification process for drug-related target genes entailed an extensive search across PharmGKB (https://www.pharmgkb.org/), the Clinical Pharmacogenetics Implementation Consortium (CPIC),and PubMed literature databases, to pinpoint common drugs and target single nucleotide polymorphisms(SNPs)for SLE. Blood samples were individually collected and genotyped using MassARRAY® high-throughput nucleic acid mass spectrometry. Genotype frequency differences were assessed through Chi-square tests against both the larger East Asian population as well as kidney transplant recipients. Data collection relied on electronic medical records, encompassing demographic details(age, gender),medication regimens(hormones, NSAIDs, hydroxychloroquine, DMARDs, biologic agents, stomach medications, calcitriol, etc.),laboratory indicators(RF, Anti-CCP antibody, ESR, CRP, anti-ANA antibodies, dsDNA antibodies, anti-SM antibodies, S m. RNP antibodies, A LT, ALB, CR, UA, WBC, PLT, HGB, Ca, K, Glu, CHOL, TG, LDL-C, HDL-C) and lupus activity scores(SLEDAI-2K). Possible disease susceptibility genes were categorized, and SPSS26 software facilitated statistical analyses., Results: The research encompassed a total of 137 SLE patients along with 50 SNPs. After conducting statistical analyses, it emerged that there existed significant disparities in CYP2D6 gene (rs1065852) distribution when compared against allele mutation rates within both East Asian populations ( p < .05) and kidney transplant patients( p < .05). Wild-type gene (GG) constituted 14% of cases while mutant gene (GA + AA) constituted 86%. Allele mutation rate (A63.6%) was significantly higher among SLE patients (RR = 0.802; p = .0355). Furthermore, the variant rs1065852 genotype (GA + AA) demonstrated significant associations with lower CRP levels, higher HGB levels, and higher HDL-C levels ( p < 0 0.05)., Conclusion: The metabolic enzyme CYP2D6 may be used as susceptibility gene for predicting systemic lupus erythematosus and are correlated with CRP and other indicators., Competing Interests: Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Objectives: There are often discrepancies in the evaluation of disease activity between patients and physicians in systemic lupus erythematosus (SLE). In this study, we examined the factors that affect those evaluations., Methods: Physician visual analogue scale (Ph-VAS), patient VAS (Pt-VAS), Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2k), glucocorticoid (GC) usage and dose, age, Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index, and three patient-reported outcomes (SLE symptom checklist [SSC], short-form 36 questionnaire [SF-36], and LupusPRO) were obtained from a study performed in 2019 using 225 SLE outpatients of the Kyoto Lupus Cohort at Kyoto University Hospital. Correlations among Ph-VAS, Pt-VAS, or dif (Pt-VAS-Ph-VAS) (Pt-VAS minus Ph-VAS) and other factors were examined., Results: We found a significant discrepancy between Pt-VAS (median 38.0 mm) and Ph-VAS (median 18.7 mm) scores ( p < 0.001). SSC score showed a significant correlation with Pt-VAS and dif (Pt-VAS-Ph-VAS) ( p < 0.001). Among SSC items, fatigue showed the most significant correlation with dif (Pt-VAS-Ph-VAS). We also showed that higher dif (Pt-VAS-Ph-VAS) was associated with lower quality of life (QOL) evaluated by SF-36 and LupusPRO., Conclusions: Pt-VAS scores tended to be higher than Ph-VAS scores, and the discrepancy was influenced mainly by fatigue. Higher dif (Pt-VAS-Ph-VAS) was associated with lower patient QOL., Competing Interests: Declaration of conflicting interestsThe author(s) declared the following potential conflicts of interest with respect to the research, authorship, and/or publication of this article: MH received research grants and/or speaker fee from Abbvie, Asahi Kasei, Astellas, Brystol Meyers, Chugai, EA Pharma, Eisai, Daiichi Sankyo, Eli Lilly, Novartis Pharma, Taisho Toyama, Tanabe Mitsubishi. K.U. received a research and/or speaker fee from Sumitomo Pharma, outside the submitted work. A.O. received a research and/or speaker fee from Daiichi-Sankyo, AbbVie G.K., Chugai Pharmaceutical Co. Ltd, Eli Lilly Japan, GSK, Mitsubishi Tanabe, Asahi Kasei Pharma and Pfizer Inc. R.W. received a research grant from AbbVie and speaker’s fee from Asahi Kasei, Chugai, Eli Lilly, and GSK. K.K. received a research and/or speaker fee from GSK K. K. R.N received a research and/or speaker fee from Takeda and Medical & Biological Laboratories Co. Ltd, Bristol-Myers Squibb, Astellas Pharma, Boehringer Ingelheim, Actelion Pharmaceuticals, and Mitsubishi Tanabe Pharma, outside the submitted work. H.Y. has received a research grant from GSK and honoraria from Chugai, unrelated to this work. R.U. received personal fees from Eisai, Sawai Pharmaceutical, Statcom, SAS Institute Japan, and EPS Corporation, outside the submitted work. M.T. received a research and/or speaker fee from AbbVie G.K., Asahi Kasei Pharma Corp., Astellas Pharma Inc., Ayumi Pharmaceutical Corp., Bristol-Myers Squibb, Chugai Pharmaceutical Co. Ltd, Eisai Co., Ltd, Eli Lilly Japan K. K., Pfizer Inc., UCB Japan Co. Ltd, Janssen Pharmaceutical K. K., Mitsubishi Tanabe Pharma Corp., Novartis Pharma K. K., Taisho Pharma Co. Ltd. K.O. received research grants and/or speaker’s fees from AbbVie, Actelion, Asahikasei Pharma, Astellas, AYUMI, Bristol-Myers Squibb, Chugai, Daiichi-Sankyo, Eisai, Eli Lilly, GSK, Janssen, JB, Mitsubishi Tanabe, Nippon Kayaku, Nippon Shinyaku, Novartis, Sanofi, and Takeda while the present study was being performed. A.M. received research grants and/or speaker’s fees from Eli Lilly Japan K.K., Ono Pharmaceutical Co., Pfizer Inc., UCB Japan, AbbVie G.K., Asahi Kasei Pharma., and Chugai Pharmaceutical Co. Ltd. M.T. and M.H. and A. O are in the endowed chair and are funded by two local governments in Japan (Nagahama City, Shiga and Toyooka City, Hyogo) and five pharmaceutical companies (Mitsubishi Tanabe Pharma Corp., Chugai Pharmaceutical Co. Ltd, Ayumi Pharmaceutical Corp., Asahi Kasei Pharma Corp., and UCB Japan Co. Ltd). H.D., Y.T., R.I., T.K., T.Y., T.I., M.T., M.T., Y.T., M.S., H.O., H.T., S.A., K.M., W.Y., and T.I. declared no conflicts of interest. The sponsors were not involved in the study design; the collection, analysis, and interpretation of data; writing this manuscript; or the decision to submit the article for publication. The authors, their immediate families, and any research foundations with which they are affiliated have not received any financial payment or other benefits from any commercial entity related to the subject of this article.

Objective: To summarize the causes of death and clinical characteristics of systemic lupus erythematosus (SLE) hospitalized patients in the last 20 years to improve SLE survival rates by detecting critical SLE early., Methods: In this case-control study, 218 SLE death cases were retrospectively analyzed from January 2002 to December 2022, with 110 SLE inpatients chosen at random as controls. The clinical symptoms, causes of death, and risk factors in patients with SLE were investigated., Results: There were 218 deaths among 9538 patients with SLE, including 188 women and 30 men. The death rate fell steadily from 4.14% in 2002 to 1.96% in 2013 and remained at 1.84% from 2014 to 2022. The standardized mortality ratio (SMR) was 4.98 [95% CI (4.06-5.89)] from 2002 to 2012 and 3.39 [95% CI (2.74-4.04)] from 2013 to 2022. Infection, lupus-induced multiple organ failure syndrome (MODS), and neuropsychiatric lupus (NPLE) were the leading causes of death, accounting for 31.19%, 15.14%, and 11.47% of overall deaths. Age had a significant association with the major causes of death. Logistic regression analysis showed NPLE[OR = 10.772,95% CI (3.350,34.633), p < 0.001], lupus pulmonary involvement (LP)[OR = 3.844,95%CI (1.547,9.552), p = 0.004], pneumonia[OR = 3.439,95%CI(1.552,7.621), p = 0.002], thrombocytopenia[OR = 14.941,95%CI (4.088,54.604), p < 0.001], creatinine>177 μmol/L[OR = 8.644,95%CI (2.831,26.388), p < 0.001], glutamic transaminase(AST) > 60U/L[OR = 5.762,95%CI (2.200,15.088), p < 0.001], total bilirubin > 34 μmol/L[OR = 16.701,95%CI (3.349,83.294), p = 0.001], higher SLE Disease Activity Index (SLEDAI)[OR = 1.089,95%CI (1.032,1.149), p = 0.002] and SLE Damage Index (SDI)[OR = 3.690,95%CI (2.487,5.474), p < 0.001] correlated positively with death., Conclusion: From 2002 to 2013, the mortality rate among patients with SLE fell steadily but remained unchanged from 2014 to 2022. Patients with SLE had significantly higher SMR than the general population. Childhood-onset SLE had a poorer prognosis than adult-onset SLE. Infection, MODS, and NPLE were the three leading causes of death. Major organ involvement and high disease activity were risk factors for mortality., Competing Interests: Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Background: Systemic lupus erythematosus (SLE) is a complex autoimmune disease with unclear etiology. Growing evidence suggests the microbiome plays a role in SLE pathogenesis. However, findings are inconsistent across studies due to factors like small sample sizes and geographical variations. A comprehensive meta-analysis is needed to elucidate microbiome alterations in SLE., Objective: This study aimed to provide a systematic overview of microbiota dysbiosis across body sites in SLE through a meta-analysis of alpha diversity indices, beta diversity indices, and abundance taxa of microbiome., Methods: A literature search was conducted across four databases to identify relevant studies comparing SLE patients and healthy controls. Extracted data encompassed alpha and beta diversity metrics, as well as bacterial, fungal, and viral abundance across gut, oral, skin, and other microbiota. Study quality was assessed using the Newcastle-Ottawa Scale. Standardized mean differences and pooled effect sizes were calculated through meta-analytical methods., Results: The analysis showed reduced alpha diversity and distinct beta diversity in SLE, particularly in the gut microbiota. Taxonomic analysis revealed compositional variations in bacteria from the gut and oral cavity. However, results for fungi, viruses, and bacteria from other sites were inconsistent due to limited studies., Conclusions: This meta-analysis offers a comprehensive perspective on microbiome dysbiosis in SLE patients across diverse body sites and taxa. The observed variations underscore the microbiome's potential role in SLE pathogenesis. Future research should address geographical variations, employ longitudinal designs, and integrate multi-omics approaches., Competing Interests: Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Introduction: Systemic Lupus Erythematosus (SLE) is often associated with antiphospholipid syndrome (APS), which manifests as recurrent thrombotic events or obstetric complications in presence of antiphospholipid antibodies. Hereby we present a case of a child who presented with low grade fever, superficial thrombophlebitis with mucosal bleeding and was diagnosed as Lupus Anticoagulant Hypoprothrombonemia Syndrome (LAHS)., Case: A 7-year-old girl was hositalized with complaints of fever and spontaneous bleeding from gums and epistaxis. On examination, she had multiple small tender nodular lesions with greenish hue of overlying skin suggesting superficial thrombophlebitis and mild non-tender hepatosplenomegaly. Her coagulogram revealed normal platelet counts and deranged PT and APTT. ESR and CRP were raised. Serology for viral infections, blood and urine cultures were negative. Patient had persistent coagulopathy, mucosal bleeding and low-grade fever despite supportive treatment. She was tested for anti-nuclear antibodies (ANA) in view of suspicion of autoimmune process. ANA was positive in high titer with speckled pattern on indirect immunofluorescence. Mixing studies showed correction of PT and non-correction of APTT. PT based factors were normal except for prothrombin (FII) which was low and remained low despite dilution. APTT based factors (FVIII and FIX) were low but corrected on dilution. This was suggestive of prothrombin deficiency and a presence of a nonspecific inhibitor of APTT pathway (likely lupus anticoagulant). Presence of antiprothrombin antibodies established the diagnosis of LAHS. ENA profile was positive for SmD1, Ro60 and Ku. Complement levels were low. Direct Coomb's test was positive but there was no evidence of hemolysis. Lupus anticoagulant by DRVVT and anti-cardiolipin antibodies by ELISA were positive. Patient was diagnosed as Systemic Lupus Erythematosus with Lupus Anticoagulant Hypoprothrombinemia Syndrome. She was treated with IV methylprednisolone. Patient showed significant improvement in form of resolution of fever, mucosal bleeding, correction of deranged INR and reversal of hypocomplementemia. She was discharged on hydroxychloroquine, mycophenolate mofetil and tapering doses of prednisolone. On follow up, child was doing well and her prothrombin time and complement levels had normalized. Low dose aspirin was aspirin was added for thromboprophylaxis., Competing Interests: Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Objective: Late-onset systemic lupus erythematosus (LoSLE) is known to possess characteristics different from those of early-onset SLE (EoSLE), thereby making their diagnosis difficult. This study aimed to assess the characteristic features of LoSLE in Japan, a model country with a super-aged society., Methods: Data were obtained from the Lupus Registry of Nationwide Institutions, which includes a multicenter cohort of patients with SLE in Japan who satisfied the 1997 American College of Rheumatology revised classification criteria for SLE. Data were compared between patients with LoSLE (≥50 years old at onset) and EoSLE (<50 years old at onset). To identify factors associated with LoSLE, binary logistic regression was used for the multivariate analysis, and missing values were complemented by multiple imputations. We also conducted a sub-analysis for patients diagnosed within 5 years of onset., Results: Out of 929 enrolled patients, 34 were excluded owing to a lack of data regarding onset age. Among the 895 remaining patients, 100 had LoSLE, whereas 795 had EoSLE. The male-to-female ratio was significantly higher in the LoSLE group than in the EoSLE group (0.32 vs 0.11, p < 0.001). With respect to SLEDAI components at onset, patients with LoSLE exhibited a higher frequency of myositis (11.9% vs 3.75%, p = 0.031), lower frequency of skin rash (33.3% vs 67.7%, p < 0.001), and lower frequency of alopecia (7.32% vs 24.7%, p = 0.012). No significant differences in overall disease activity at onset were observed between the two groups. Regarding medical history, immunosuppressants were more commonly used in EoSLE. A multivariate analysis revealed that a higher male proportion and a lower proportion of new rash at onset were independent characteristic features of LoSLE. We also identified late onset as an independent risk factor for a high SDI score at enrollment and replicated the result in a sub-analysis for the population with a shorter time since onset., Conclusions: We clarified that LoSLE was characterized by a higher male proportion, a lower frequency of skin rash and a tendency to organ damage. Now that the world is faced with aging, our results may be helpful at diagnosis of LoSLE., Competing Interests: Declaration of conflicting interestsThe author(s) declared the following potential conflicts of interest with respect to the research, authorship, and/or publication of this article: Ken-ei Sada received a speaker’s fee from GlaxoSmithKline PLC and research grants from Pfizer Inc. Yohei Kirino received a speaker’s fee from Amgen and Novartis and research funding from Nippon Shinyaku. Ryusuke Yoshimi received a speaker’s fee from GlaxoSmithKline PLC, AstraZeneca PLC, and Sanofi S.A.

Introduction: Medication nonadherence is common in systemic lupus erythematosus (SLE) and associated with morbidity and mortality. We explored the reliability of pharmacy data within the electronic medical record (EMR) to examine factors associated with nonadherence to SLE medications., Methods: We included patients with SLE who were prescribed ≥1 SLE medication for ≥90 days. We compared two datasets of pharmacy fill data, one within the EMR and another from the vendor who obtained this information from pharmacies and prescription benefit managers. Adherence was defined by medication possession ratio (MPR) ≥80%. In addition to MPR for each SLE medication, we evaluated the weighted-average MPR and the proportion of patients adherent to ≥1 SLE medication and to all SLE medications. We used logistic regression to examine factors associated with adherence., Results: Among 181 patients (median age 36, 96% female, 58% Black), 98% were prescribed hydroxychloroquine, 34% azathioprine, 33% mycophenolate, 18% methotrexate, and 7% belimumab. Among 1276 pharmacy records, 74% overlapped between linked EMR-pharmacy data and data obtained directly from the vendor. Only 9% were available from the vendor but not through linked EMR-pharmacy data. The weighted-average MPR was 57%; 45% were adherent to hydroxychloroquine, 46% to ≥1 SLE medication, and 32% to all SLE medications. Older age was associated with adherence in univariable and multivariable analyses., Discussion: Our study showed that obtaining linked EMR-pharmacy data is feasible with minimal missing data and can be leveraged in future adherence research. Younger patients were more likely to be nonadherent and may benefit from targeted intervention., Competing Interests: Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Background: Systemic lupus erythematosus is a multisystemic rheumatic disease with different clinical features. Disturbance in apoptosis regulation seems to be a major factor in SLE development., Objective: Survivin plays a key role in mitosis and inhibiting apoptosis. A study was conducted to examine the expression level of survivin and miRNAs that affect survivin transcript levels in patients with SLE., Methods: We isolated peripheral blood mononuclear cells from 50 inactive SLE patients and 50 healthy controls. RNA is extracted and converted to cDNA. The quantitative real-time polymerase chain reaction is conducted to assess the expression levels of survivin total and its variants with effective miRNAs in PBMCs., Results: Expression levels of miR-34a-5p (fold change = 1.5, p + + = 0.027), and 218-5p (fold change = 1.5, p + + = 0.020) were significantly increased. While miR-150-5p (fold change = 0.56, p + + = 0.003) was significantly decreased. The mRNA expression of survivin-WT (fold change = 0.63, p + + = 0.002) was significantly downregulated in SLE patients compared to the healthy controls. Survivin total and its two major variants (survivin-2B, and survivin-ΔEx3) did not differ significantly between SLE patients and controls., Conclusion: Although survivin-TS and its two variants (survivin-2B, and survivin-ΔEx3) were not differently expressed in SLE patients, survivin-WT had altered expression. Despite aberrant miRNA expression in PBMCs from SLE patients, survivin and miRNA expression were not associated with leukopenia. The pathogenesis of SLE disorder might be linked to survivin's other roles in the immune system aside from anti-apoptotic functions., Competing Interests: Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Objective: Systemic lupus erythematosus (SLE) constitutes an autoimmune disorder with potential involvement of the gastrointestinal system (GIS). Our objective was to assess the gastrointestinal (GI) manifestations in patients diagnosed with childhood onset SLE., Methods: The study cohort consisted of 123 patients with childhood onset-SLE and GIS involvement from 16 referral departments of pediatric rheumatology. All participants met the Systemic Lupus International Collaborating Clinics criteria., Results: Out of 123 patients, 78 (63.4%) exhibited GIS involvement at the initial SLE diagnosis, whereas the remaining 45 (36.6%) developed GI symptoms after a median duration of 12 (3-140) months. Eighty-two (66.7%) individuals experienced symptoms related to the GI tract, whereas the remaining patients received a diagnosis of GI involvement through laboratory assessments. The predominant initial GIS involvement symptom was abdominal pain, observed in 77 (62.6%) patients, followed by elevated hepatic transaminases in 70 (56.9%), hepatomegaly in 40 (32.5%), diarrhea in 26 (21.1%), and jaundice in 11 (8.9%) patients. The GIS involvement was associated with SLE in 82 (78.6%), while it resulted from drug-related adverse events in 35 (28.5%) patients or comorbidities in 6 (0.5%) patients., Conclusion: GIS involvement should be considered in all childhood onset-SLE patients, especially in the presence of suggestive symptoms or elevated hepatic transaminases. It is also crucial to consider SLE in the differential diagnosis of GIS manifestations in children. Apart from GIS involvement directly associated with SLE, adverse events of drugs should be kept in mind., Competing Interests: Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Background: The exact cause of systemic lupus erythematosus (SLE) is still unknown. However, hormonal, genetic, and environmental factors may play significant roles in its development. Infection has been recognized as a crucial trigger for SLE development. Several studies have reported a higher prevalence of Toxoplasma gondii infections in patients with SLE than in healthy individuals. However, these results were inconsistent. Therefore, this study aimed to conduct a systematic review and meta-analysis of published studies to provide a definitive conclusion regarding the relationship between T. gondii infection and SLE., Materials and Methods: We conducted a comprehensive search across diverse databases using an array of search tools to uncover pertinent literature. Following the stringent application of the inclusion and exclusion criteria, we carefully selected the appropriate reports for our meta-analysis. Using Comprehensive Meta-Analysis software v4, we analyzed the data and determined the prevalence of antibodies against T. gondii in patients affected with SLE. To investigate the correlation between T. gondii seropositivity and SLE, we computed the risk ratios (RRs) and 95% confidence intervals (CI)., Results: Eleven studies were considered eligible for inclusion in the present study. The prevalence of anti-IgG and IgM antibodies against T. gondii was 33.9% and 7.7%, respectively. A significant association between T. gondii IgG seropositivity and SLE was observed when compared to the controls (risk ratio = 2.14, 95% CI = 1.42 to 3.22, p = .000). However, IgM seropositivity against T. gondii was comparable between patients with SLE and healthy controls., Conclusions: In summary, this study suggests that T. gondii IgG is more prevalent in patients with SLE than in healthy individuals in areas where T. gondii infections are more frequent. However, an exact cause-and-effect relationship still needs to be established. Therefore, additional research is necessary to validate these findings and to investigate the underlying mechanisms., Competing Interests: Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Background: The soluble CD163 (sCD163) was elevated in systemic lupus erythematosus (SLE) patients., Purpose: To study whether serum sCD163 could be used to predict the occurrence and prognosis of lupus nephritis (LN)., Research Design: The recruited patients were classified into different groups according to standard identification criteria., Study Sample: The patients with LN., Data Collection and Analysis: 11 indices were analyzed and compared in SLE and LN patients. Furthermore, the level of serum sCD163 was detected using an enzyme-linked immunosorbent assay. Meanwhile, the receiver operating characteristic analysis was performed to evaluate the prediction effect of sCD163. Additionally, spearman correlation analysis of serum sCD163 with indices was conducted., Results: There were six positive indices and one negative risk factor correlated to LN. sCD163 was elevated in LN patients and could be used to diagnose LN. Importantly, sCD163 was increased in LN patients with a heavy SLE disease activity index. Finally, it was revealed that the level of sCD163 was higher in the LN patients with no response than that with complete or partial response, which also could predict the prognosis of LN., Conclusions: Serum sCD163 was elevated in LN patients than in SLE patients, which could be used to predict the occurrence and prognosis of LN., Competing Interests: Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Background: Systemic lupus erythematosus (SLE) is a chronic inflammatory multisystemic disease. Monitoring disease activity thoughtout the disease course is important for effective management and assessment of disease outcome., Objective: To assess whether the pan-immune inflammation value (PIIV) at diagnosis could predict organ involvement and disease activity in childhood SLE (cSLE) patients after 12 months of disease onst., Methods: This is an observational retrospective multicenter study that comprised cSLE patients seen and followed at the participating centers between January 2010 and December 2022. All patients met the EULAR/ACR-19 criteria, were immunosuppressive drug-naïve at the time of SLE diagnosis and had a minimal follow-up period of 12 months. The data included clinical and laboratory findings and disease activity using the SLEDAI-2K. Receiver operating characteristic (ROC) curves were employed to determine the optimal cut-off value of PIIV and assess its predictive potential for disease activity, and organ involvement., Results: A total of 125 patients (104 female) with a median age of 16.0 (IQR 5.6) years, a median age at disease onset of 10.9 (IQR 3.0) years, and a median disease duration of 4.8 (IQR 5.3) years were included. The most frequent involved organs at diagnosis were hematological (89.6%), musculoskeletal (68.8%), mucocutaneous (63.2%), and renal (58.4%). However, at a 12-month follow-up visit, the most frequent involved organs were renal (40.0%), hematological (39.2%), musculoskeletal (15.2%), and mucocutaneous (10.4%). The median PIIV at diagnosis was 139 (IQR 229.6), while the median SLEDAI was 12 (IQR 6.5) and 3.5 (IQR 7.0) at diagnosis and 12 months, respectively. An optimal PIIV cut-off of 250 was found to be a predicative for disease activity, with a sensitivity of 45% and a specificity of 86%. The study revealed that the PIIV successfully predicted four systems in our cohort of patients., Conclusion: Our work suggests the PIIV might be a reasonable predictor for organ involvement and disease activity in newly diagnosed cSLE, though further research, particularly larger studies, is required to validate these findings, especially regarding organ involvement., Competing Interests: Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Background: Systemic lupus erythematosus is a chronic autoimmune inflammatory disease characterized by multiple symptoms. The phenolic acids and other flavonoids in Nelumbo nucifera have anti-oxidants, anti-inflammatory, and immunomodulatory activities that are essential for managing SLE through natural sources. This study employs network pharmacology to unveil the multi-target and multi-pathway mechanisms of Nelumbo nucifera as a complementary therapy. The findings are validated through molecular modeling, which includes molecular docking followed by a molecular dynamics study., Methods: Active compounds and targets of SLE were obtained from IMPPAT, KNApAcKFamily and SwissTargetPrediction databases. SLE-related targets were retrieved from GeneCards and OMIM databases. A protein-protein interaction (PPI) network was built to screen out the core targets using Cytoscape software. ShinyGO was used for GO and KEGG pathway enrichment analyses. Interactions between potential targets and active compounds were assessed by molecular docking and molecular dynamics simulation study., Results: In total, 12 active compounds and 1190 targets of N. nucifera's were identified. A network analysis of the PPI network revealed 10 core targets. GO and KEGG pathway enrichment analyses indicated that the effects of N. nucifera are mediated mainly by AGE-RAGE and other associated signalling pathways. Molecular docking indicated favourable binding affinities, particularly leucocianidol exhibiting less than -4.5 kcal/mol for all 10 targets. Subsequent molecular dynamics simulations of the leucocianidol-ESR1 complex aimed to elucidate the optimal binding complex's stability and flexibility., Conclusions: Our study unveiled the potential therapeutic mechanism of N. nucifera in managing SLE. These findings provide insights for subsequent experimental validation and open up new avenues for further research in this field., Competing Interests: Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Objective: Skin involvement is common in systemic lupus erythematosus (SLE), but may be resistant to conventional treatment. We sought to evaluate the efficacy of anifrolumab (ANI) in refractory cutaneous manifestations of SLE., Methods: Case series of patients with refractory cutaneous SLE from three Rheumatology Departments in Greece. Outcome measures were improvement in Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K), physician global assessment (PGA) and Cutaneous Lupus Erythematosus Disease Area and Severity Index (CLASI). Clinically relevant improvement in skin was defined as decrease ≥50% (CLASI50) from baseline values., Results: Eighteen patients received ANI; all had active skin involvement at baseline. Mean (SD) SLEDAI and PGA at ANI initiation were 7.4 (2.7) and 1.4 (0.5), respectively, with a mean prednisone dose 4.9 (4.5) mg/day. Mean CLASI (Activity/Damage) at baseline was 13.9 (9.7)/2.9 (4.6). Patients were refractory to a mean 6.3 (1.5) immunomodulatory agents (including hydroxychloroquine and glucocorticoids) before the initiation of ANI. After a mean 8.5 (4.6) months, 89% ( n = 16/18) of patients demonstrated significant improvement in general lupus and cutaneous disease activity, and glucocorticoid tapering. Mean SLEDAI and mean CLASI at last visit were 3.4 (1.9) and 2.1 (2.4)/1.4 (2.2), respectively, and mean daily prednisone dose decreased to 2.4 (2.2). Of note, in this group of highly refractory patients CLASI50 was achieved in 16/18 (89%) patients. One patient discontinued ANI after 4 infusions due to a varicella-zoster virus infection and one patient, who initially responded to treatment with ANI, experienced a skin flare due to temporary discontinuation due to Covid 19 infection. DORIS remission and LLDAS were attained in two (11.1%) and eleven (61.1%) patients, respectively., Conclusion: Anifrolumab is highly effective in various skin manifestations of SLE, even after prior failure to multiple treatments., Competing Interests: Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Background: Systemic lupus erythematosus (SLE) is a systemic autoimmune disease with multi organ involvement. One of the most common manifestations is pulmonary disease with a reported prevalence between 5%-90%., Purpose: Given this wide range of prevalence, there is a need to more closely define types of pulmonary disease in SLE and associated risk factors., Research Design: We sought to characterize the presentation of pulmonary manifestations in an established SLE cohort using electronic health record data., Study Sample: All patients were >18 years of age and had confirmed SLE by a rheumatologist using SLICC or 2019 ACR/EULAR classification criteria. 220 patients with imaging were included in this study; average age was 42.5 years, 86.7% identified as female, 60.5% identified as white, 37.3% as Black, and 1.82% as Asian., Analysis: Generalized estimating equations were utilized to analyze the data, accounting for its repeated measured nature., Results: We found an association between smoking (present/prior smoker) and radiologist reported disease on computerized tomography (CT) scan, as well as an association between smoking (present/prior smoker), older age, and male sex with having pulmonary disease identified on chest X-ray. The most common findings on CT and X-ray were increased lung density (24%, 12%) and atelectasis (18%, 10%). The most common disease found on CT was pleural effusion (24%) and mediastinal/axillary lymphadenopathy (16%)., Conclusion: While our study is limited by the retrospective nature, our results show that certain factors, namely smoking, older age, or male sex should prompt clinicians to have a higher suspicion for lung disease in SLE patients., Competing Interests: Declaration of conflicting interestsThe author(s) declared the following potential conflicts of interest with respect to the research, authorship, and/or publication of this article: Emily Littlejohn is a paid speaker for Aurinia Pharmaceuticals and consultant for GSK.

Objectives: This study aims to explore the treatment pattern of systemic lupus erythematosus (SLE) in Aotearoa/New Zealand., Methods: SLE patients were linked to the pharmaceutical dispensing data. The use of publicly funded anti-malarials, immunomodulators, biologics, glucocorticoids and bisphosphonates were compared by gender, ethnicity, age group, socioeconomic status and year of SLE identification. Adherence to hydroxychloroquine was examined using the medication possession ratio (MPR), with a MPR of ≥0.8 considered as high adherence., Results: Of the 2631 SLE patients, 73.8% used hydroxychloroquine, 64.1% used immunomodulators/biologics and 68.0% used 5 mg or more prednisone daily for at least 90 days. Women were more likely to use hydroxychloroquine than men. Asian patients had a different treatment pattern than other ethnic groups, and Māori were less likely to use hydroxychloroquine. The proportions of patients using different treatments decreased with age. Of the patients using hydroxychloroquine, 54.5% had high adherence. For patients over 40 years old and on long term prednisone, 47.3% had bisphosphonates and this figure was 17.8% for patients under the age of 40 years old. Patients with better socioeconomic status had a higher probability of using bisphosphonates than patients with lower socioeconomic status., Conclusions: Adherence to hydroxychloroquine in these patients varied and was lower in men and in Māori. Prednisone is commonly prescribed and used long term. Half of those over the age of 40 years old co-administered bisphosphonate. Further research is needed to identify the reasons for these discrepancies on SLE treatments by gender, ethnicity, age and socioeconomic status., Competing Interests: Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Background: Physical inactivity, which is highly prevalent in patients with systemic lupus erythematosus (SLE), is an independent risk factor for cardiovascular events and causes many complications. This study aimed to investigate the effect of objective measurement and physical activity level on peripheral muscle strength, exercise capacity, pain, dyspnea, fatigue, anxiety, and depression in patients with SLE., Methods: The present cross-sectional study analyzed 41 patients with SLE. Clinical and demographic characteristics of patients were recorded. Functional exercise capacity, peripheral muscle strength, dyspnea, pain, fatigue, anxiety, and depression were assessed. The physical activity level was assessed by a wearable activity tracker (Mi Band four smart band)., Results: The number of steps measured by the activity tracker was 4384.43 ± 1558.21 steps per day in patients with SLE. Patients with physical activity levels below 5000 steps exhibited elevated levels of fatigue, along with diminished functional exercise capacity and knee muscle strength, in comparison to those who were above the 5000-step threshold. Physical activity levels correlated with functional exercise capacity (6MWT), physiological parameters (maximum heart rate, Δ heart rate, Δ dyspnea, QFM fatigue, Δ QFM fatigue), and knee extension muscle strength. The functional exercise capacity and knee extension were identified as significantly and dependently associated with physical activity levels in SLE patients., Conclusion: Physical activity level is associated with functional exercise capacity and knee muscle strength in patients with SLE., Competing Interests: Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Introduction: Anti-neuronal antibodies target antigens produced by tumour cells and cells of nervous system. These antibodies are formed as a result of autoimmune response elicited by the underlying malignancy, when proteins restricted to immune privileged neurons are presented by the tumour. Previous studies have shown presence of anti-neuronal antibodies in systemic lupus erythematosus and neuropsychiatric lupus (NPSLE) but information on individual antibodies and their pathogenic role is lacking., Aims/objective: To assess the frequency of anti-neuronal antibodies in our neuropsychiatric lupus cohort and to assess any significant association with specific neurological syndrome and to see if the antibodies were more likely to occur in active rather than inactive neuropsychiatric lupus., Methodology: This cross-sectional study was conducted in our center from 2019 to 2022. Neuropsychiatric manifestations were defined according to 1999 American College of Rheumatology (ACR) nomenclature and case definitions for neuropsychiatric lupus. Samples were taken from active or inactive NPSLE patients with their informed consent. Testing was done on an anti-neuronal antigen panel which consisted of [Amphiphysin, CV2, GAD 65, PNMA2 (Ma-2/Ta), Ri, Yo, Hu, recoverin, SOX1, titin, Zic, Tr)] by semi-quantitative Line immune assay. Association between the categorical variables and antibody positivity group was established using chi-square/Fisher's exact test as appropriate., Results: 65 patients were recruited, of which 23 (35%) patients had active NPSLE at the time of sample collection. Anti-neuronal antibodies were positive in 13/65 (20%) patients with anti-Gad 65 antibodies having the highest frequency (6.2%) followed by anti CV 2 (3.1%), anti Sox1 (3.1%), anti Amphiphysin (3.1%) anti recoverin (1.5%), anti Yo (1.5%) and anti Zic (1.5%). The panel of anti-neuronal antibodies did not show any specific association with NPSLE features.However, an interesting finding was that, patients with active disease had higher odds of having anti-neuronal antibodies with an OR = 10 (95% CI:2.38 -42) ( p < 0.001) than inactive disease., Conclusion: Anti-neuronal antibodies were more likely to be positive in active neuropsychiatric lupus patients, and these antibodies which are commonly used to diagnose paraneoplastic syndromes may have a potential role in the diagnosis of NPSLE., Competing Interests: Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Systemic lupus erythematosus (SLE) patients are susceptible to marantic endocarditis (ME) due to a hypercoagulable state. The literature regarding the epidemiology and outcomes of ME in SLE patients is limited. All patients ≥18 years who had SLE with and without ME between 2007 and 2019 were identified from the National Inpatient Sample in the United States (US). Predictors of inpatient mortality for SLE patients with ME were analyzed. Between 2007 and 2019, there were 508,818 hospitalizations for SLE, of which 785 (0.2%) had ME. Of SLE patients with ME, 33 (4.2%) died while hospitalized over the study period. On multivariate analysis, female sex (adjusted odds ratio (aOR), 95% confidence intervals: 24.72 (3.21, 190.27)), age <34 years (aOR: 6.81 (1.80, 25.79)), anemia (aOR: 3.41 (1.12, 10.40)), antiphospholipid syndrome (aOR: 13.50 (3.83, 47.64)), stroke complicating ME (aOR: 9.64 (3.24, 28.71)), and acute kidney injury (aOR: 3.74 (1.06, 13.20)) were all associated with increased inpatient mortality among SLE patients with ME ( P < .05 for all). Between 2007 to 2019, ME occurred in 0.2% of SLE hospitalizations, with a 4.2% average inpatient mortality over the study period. Female sex, antiphospholipid syndrome, and stroke were most strongly associated with increased inpatient mortality., Competing Interests: Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Background: Although high serum levels of interleukin (IL)-17 and its producing cells have been found in rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE) in earlier research, it is still unclear how these findings relate to disease activity., Objectives: This study examines the link between serum levels of IL-17 and the activity of both RA and SLE., Design: This pilot case-control study included 100 patients with RA, 100 with SLE, and 100 healthy controls., Methods: The Disease Activity Score-28 (DAS28) scores assessed the activity of RA, whereas the Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) scores assessed SLE activity. All participants' data were compared and correlated., Results: Serum levels of IL-17 were significantly higher in RA and SLE patients compared with the controls ( P  < .001) and showed significantly positive correlations ( P  < .001) with rheumatoid factor titer, anti-cyclic citrullinated peptide (anti-CCP) and DAS28 score among the RA patients. Although among SLE patients, they were significantly positively correlated ( P  < .001) with anti-double-stranded DNA (anti-ds DNA) levels and the SLEDAI-2K scores, the best cut-off value of IL-17 for predicting moderate and high disease activity was > 175 pg/mL among RA patients and > 95 pg/mL among SLE patients., Conclusions: There is a significant correlation between RA and SLE activity and serum levels of IL-17. This discovery emphasizes IL-17 as a potential therapeutic target., Competing Interests: The authors declare that there is no conflict of interest., (© The Author(s) 2024.)

Context: Cognitive deficits are neuropsychiatric syndromes associated with systemic lupus erythematosus. In our context, there are no data on the frequency of cognitive deficit as a manifestation of neuropsychiatric SLE or the associated conditions., Objective: To define determinants of cognitive deficit in a cohort of Colombian patients with SLE attending a third-level hospital., Methods and Patients: This descriptive cross-sectional study included patients with SLE, explored the presence of cognitive impairment through screening testing using the Montreal Cognitive Assessment (MoCA test), and diagnostic confirmation with a specific neuropsychological test battery recommended by the American College of Rheumatology. Quality of life was assessed using the LupusCol questionnaire and depression using the Beck Depression Inventory., Results: Most patients were women, with a median age of 37 years (IQR, 28.0 - 46.7). Most patients had a level of higher education or technical education. Fifty-nine (62.9%) patients presented with a normal MoCA test result ≥26 points, and 35 (37.1%) patients with a score <26 points that were considered abnormal. The comprehensive neuropsychological test battery was applied to 31 patients (33.0%) with an abnormal MoCA test. Forty-one patients (48.8%) had some degree of depression. The median loss of quality of life was 21.03% (IQR 10.2 - 40.3). 19 patients (20%) presented some degree of cognitive deficit, 15 (15.95% of the total sample) had cognitive impairment, and 4 (4.25%) had cognitive decline. In a logistic regression analysis using data from patients undergoing specific tests, variables related to cognitive deterioration were found to be associated with a lower quality of life, showing an adjusted odds ratio of 1.05 (CI 1.01-0.09). No association was demonstrated with SLEDAI, prednisolone use, cyclophosphamide use, and the presence of depression., Conclusion: In this study, it was found in 16% of patients evaluated with the complete neuropsychological test battery and in 37% with the MoCA screening test. Our results suggest that it is crucial to implement strategies to assess cognitive deficit, depression, and quality of life in the consultation of patients with SLE and to raise awareness among health providers who care for patients with lupus about their presence and impact., Competing Interests: Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Background: Stress has been linked to worsening symptoms and increased disease activity in patients with Systemic lupus erythematosus (SLE). Life-events are individual stress points, and there is conflicting evidence regarding their role in SLE activity and disease perception., Methods: Adult SLE patients were recruited for the study. Clinical and laboratory features of SLE were recorded, and previous diagnosis of anxiety or depression were retrieved from patients' electronic charts. Flares were defined by the Systemic Lupus Erythematosus Disease Activity (SLEDAI) flare Index, and flares during the previous year were documented. During a routine visit, they completed validated Portuguese translations of the 10-item Perceived Stress Scale (PSS-10), Hospital Anxiety and Depression Scale (HADS) and Life Experience Survey (LES) for the previous year., Results: A total of 47 female SLE patients were recruited. Ten patients (21.3%) had experienced recent flares. Patients with recent flares reported fewer life events, with lower positive, negative, and total weightings sums compared to those without recent flares. Although 42.2% of patients perceived pathological levels of stress in the previous month, 48.9% had anxiety symptoms, and 34% were at high risk for an anxiety disorder, these psychometric measures did not differ significantly between the recent flare and no-flare groups., Conclusions: There is a high prevalence of pathological levels of stress among SLE patients. SLE patients with recent flares report less psychological impact from life events, both positive and negative, independent of other psychological or pharmacological factors., Competing Interests: Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.