Incidence Of SLE Research Articles

Ultra-Processed Food Intake and Risk of Systemic Lupus Erythematosus among Women followed in the Nurses' Health Study Cohorts.

We assessed ultra-processed food (UPF) intake and SLE incidence within the prospective Nurses' Health Study cohorts. 204,175 women were followed, NHS: 1984 - 2016; NHSII: 1991 - 2017. Semi-quantitative food frequency questionnaires were completed every 2-4 years. UPF intake was determined as per the Nova classification. Nurses self-reported new doctor-diagnosed SLE, confirmed by medical records. Time-varying Cox regressions estimated hazard ratios (HR, 95% confidence intervals) for incident SLE and SLE by anti-double stranded DNA antibody (dsDNA) at diagnosis, according to cumulatively-updated daily: a) UPF servings, b) total intake (gms + mls), and c) % total intake. Analyses adjusted for age, race, cohort, caloric and alcohol intakes, household income, smoking, body mass index (BMI), physical activity, menarchal age, and oral contraceptive use. We tested for interaction with BMI and examined UPF categories. Mean baseline age was ~50 years (NHS) and ~36 years (NHSII); 93% self-reported White race. 212 incident SLE cases were identified. SLE risk was higher in 3rd vs. 1st UPF tertile (servings/day pooled multivariable [MV] HR 1.56 (1.04-2.32); p trend 0.03). Results were stronger for dsDNA+ SLE (servings/day pooled MV HR 2.05 (1.15-3.65); p trend 0.01), and for absolute (servings or total) than % total intake. Sugar/artificially-sweetened beverages were associated with SLE risk (3rd vs. 1st tertile MV HR 1.45 (1.01-2.09). No BMI interactions were observed. Higher cumulative-average daily UPF intake was associated with >50% increased SLE risk, and with doubled risk for anti-dsDNA+ SLE. Many deleterious effects on systemic inflammation and immunity are postulated.

Optimal exposure of mycophenolic acid for induction therapy of childhood lupus nephritis patients: an observational cohort study.

Mycophenolic acid (MPA) is recommended for lupus nephritis (LN) treatment, but with large inter-individual variability in pharmacokinetics (PK). The aim of this study is to reveal the relationship between MPA exposure and disease response and adverse drug reactions in pediatric LN patients. This was a population-based observational cohort study. A total of 86 pediatric LN patients treated with mycophenolate mofetil (MMF) for induction therapy were enrolled. The area-under the concentration-time curve (AUC) was calculated using MPA concentrations according to a limited sampling strategy. Receiver operating characteristic analysis was performed to assess the MPA-AUC threshold values. The cumulative incidence of renal remission and inactive systemic lupus erythematosus (SLE) over time was evaluated by Kaplan-Meier's analysis. MPA-AUC was identified as an independent factor associated with renal remission and lupus activity at 6 and 12 months after MMF treatment, and the improved renal remission rates were correlated with higher MPA-AUC, with thresholds of 29.81 and 30.63 μg·h·mL-1 at 6months and 12 months, respectively. Furthermore, the thresholds for maintaining the hypoactive state of LN were 30.96 and 31.19 μg·h·mL-1at 6 months and 12 months, respectively. Patients reaching target thresholds for MPA-AUC achieved renal response or stable disease earlier. In addition, the MPA-AUC threshold for decreasing MMF-related adverse reactions was 50.80 μg·h·mL-1. The initial and long-term treatments of pediatric LN patients with MMF should be individualized according to the MPA-AUC, and the recommended MPA exposure is 31.19-50.80 μg·h·mL-1.

Epidemiology of SLE in Italy: an observational study using a primary care database

ObjectivesTo estimate the incidence and prevalence of SLE in Italy, and to describe the demographic and clinical characteristics of patients with newly diagnosed SLE.MethodsA retrospective cohort study was conducted using...

The role of vitamin D in pediatric systemic lupus erythematosus - a double pawn in the immune and microbial balance.

Having increased popularity during the Covid-19 pandemic, vitamin D3 is currently impressing thanks to the numerous researches aimed at its interactions with the body's homeostasis. At the same time, there is a peak in terms of recommendations for supplementation with it. Some of the studies focus on the link between autoimmune diseases and nutritional deficiencies, especially vitamin D3. Since the specialized literature aimed at children (patients between 0-18 years old) is far from equal to the informational diversity of the adult-centered branch, this review aims to bring up to date the relationship between the microbial and nutritional balance and the activity of pediatric systemic lupus erythematosus (pSLE). The desired practical purpose resides in a better understanding and an adequate, individualized management of the affected persons to reduce morbidity. The center of the summary is to establish the impact of hypovitaminosis D in the development and evolution of pediatric lupus erythematosus. We will address aspects related to the two entities of the impact played by vitamin D3 in the pathophysiological cascade of lupus, but also the risk of toxicity and its effects when the deficiency is over supplemented (hypervitaminosis D). We will debate the relationship of hypovitaminosis D with the modulation of immune function, the potentiation of inflammatory processes, the increase of oxidative stress, the perfusion of cognitive brain areas, the seasonal incidence of SLE and its severity. Finally, we review current knowledge, post-pandemic, regarding the hypovitaminosis D - pSLE relationship.

Declining Incidence of Systemic Lupus Erythematosus in Norway 1999-2017: Data From a Population Cohort Identified by International Classification of Diseases, 10th Revision Code and Verified by Classification.

The goal of this study was to provide complete, robust data on annual systemic lupus erythematosus (SLE) incidence rates over nearly two decades from the Southeast Norway area (2.9 million inhabitants) and assess accuracy of SLE-specific International Classification of Diseases (ICD) codes for SLE diagnosis. From administrative databases, we identified all cases International Statistical Classification of Diseases and Related Health Problems, Tenth Revision (ICD-10) coded as SLE during 1999 through 2017 in Southeast Norway. We manually reviewed the chart of every case ICD-10 coded as SLE to either confirm or reject SLE diagnosis. Using SLE classification criteria, we classified all cases with confirmed SLE. We estimated annual incidence rates of classified SLE, and subsets, defined by age at diagnosis, sex, and parental country of birth. The chi-square test was applied for linear time-trend analyses of incidence. Among the 3,488 cases ICD-10 coded as SLE, chart reviews confirmed SLE diagnosis in 1,558 (45%), of which 797 had new-onset disease during 1999 through 2017. Annual SLE incidence rates fell during 1999 to 2017. The fall was most pronounced in female persons 50 to 59 years old at diagnosis, in whom incidence fell from 3.4 to 1.1 per 100,000 persons (P trend < 0.001). Concurrent ecological data from the study area showed a 74% reduction in prescriptions of menopausal hormone treatment. Accuracy of ICD-10 codes for incident SLE diagnosis was acceptable in juveniles and young adults (up to 20 years) but otherwise low. In a presumably complete population-based cohort, we identified decreasing incidence of SLE, especially among female persons 50 to 59 years old. Although reasons for declining incidence are not clear, ecological data indicate a possible role of environmental factors, for example, menopausal hormone treatments.

GENE EXPRESSION AND SINGLE NUCLEOTIDE POLYMORPHISM (rs1140713) OF MICRORNA-126

MicroRNAs are non-coding gene regulators that may serve as systemic lupus erythematosus biomarkers for diagnosis or prognosis. This study was aimed to identify the single nucleotide polymorphism (rs1140713) of microRNA-126 and its expression by using PCR and qRT-PCR techniques. This research involved 100 womenwith SLE with a mean age of 32.85±9.992 years they were obtained from Baghdad city (medical city, Baghdad Hospital)/Iraq, along with 100 healthy controls with a mean age of 31.68±8.308 years. The mean score on the SLE disease activity index (SLEDAI-2k) was 10.860.±3.275 and a disease duration was 9.00 years, (Erythrocyte sedimentation rate, C-reactive protein, urea, and creatinine were all significantly higher in SLE patients compared to controls, while haemoglobin, white blood cells, complement 3, and complement 4 were significantly lower in contrast to controls (p-value=&lt; 0.0001**)). According to the examination of anti-nuclear antibodies (ANA) in patients, 89% of them have ANA and 95% have anti-double strand DNA (anti-dsDNA) antibodies. Single nucleotide polymorphism analysis for subjects involved in this study revealed a significant increased risk association between miRNA-126 (rs1140713) heterozygote (CT)(OR=20.64; p≤0.001) and homozygote mutant (TT) (OR = 16.35; p= 0.001) genotypes and SLE incidence. Moreover, the results showed a substantial decline in microRNA-126 expression with fold change mean (0.639±2.931) in comparison to controls' fold change mean (1.00±0.00). The present investigation found no indication of a relationship between changes in microRNA-126 folds and demographic or laboratory studies of the disease. The results suggest the microRNA-126 might consider as biomarkers in SLE pathogenesis.

Multimorbidity in systemic lupus erythematosus in a population-based cohort: the Lupus Midwest Network.

The objectives of this study were to assess the prevalence and incidence of multimorbidity and its association with the SLICC/ACR damage index (SDI) among patients with SLE. Using prevalent and incident population-based cohorts of patients with SLE and their matched comparators, we assessed 57 chronic conditions. Chronic conditions were categorized as SDI-related or SDI-unrelated. Multimorbidity was defined as the presence of two or more chronic conditions. The prevalence of multimorbidity for both cohorts was compared using logistic regression. Cox models were used to examine the development of multimorbidity after SLE incidence. The prevalent cohort included 449 patients with established SLE on 1 January 2015. They were three times more likely to have multimorbidity compared with non-SLE comparators [odds ratio (OR) 2.98, 95% CI 2.18-4.11]. The incident cohort included 270 patients with new-onset SLE. At SLE incidence, patients with SLE were more likely to have multimorbidity than comparators (OR 2.27, 95% CI 1.59-3.27). After incidence, the risk of developing multimorbidity was 2-fold higher among patients with SLE than among comparators [hazard ratio (HR) 2.11, 95% CI 1.59-2.80]. The development of multimorbidity was higher in patients with SLE based on SDI-related conditions (HR 2.91, 95% CI 2.17-3.88) than on SDI-unrelated conditions (HR 1.73, 95% CI, 1.32-2.26). Patients with SLE had a higher burden of multimorbidity, even before the onset of the disease. The risk disparity continued after SLE classification and was also seen in a prevalent SLE cohort. Multimorbidity was driven both by SDI-related and unrelated conditions.

A CASE OF SYSTEMIC LUPUS ERYTHEMATOSUS FOR CAESEREAN SECTION

Introduction : Systemic Lupus Erythematosus is a chronic multisystem disorder characterized by formation of autoantibodies against nuclear , cytoplasmic and cell membrane antigens . Most common in women of child bearing age group . Disease exacerbation , neonatal lupus , fetal loss and increased incidence of pre-eclampsia are major challenges. Case Report : A 26 year old woman G3P1L0A1D1 with 39 weeks amenorrhea with known history of SLE since 2 years scheduled for elective LSCS . She has bad obstetric history and on medication with Tab.Azathioprine 50mg OD , Tab.Hydroxychloroquine 200mg OD and Tab.Prednisolone 5mg BD. ANA antibodies were found to be positive but antiphospholipid antibodies were negative. Regional anaesthesia preferred over general anaesthesia because of anticipated difcult airway. Subarachnoid block performed using 2ml of 0.5% bupivacaine heavy . Surgery uneventful with minimum blood loss. Discussion ;The peak incidence of SLE occurs between the ages of 15 and 40 years, with an estimated female-to-male incidence of 9:1. It is characterized by autoantibody production and dysfunctional immune system resulting in organ inammation andconsequent damageSLE may be associated with secondary APS which is a multisystem disorder characterized by recurrent systemic arterial and venous thrombosis, recurrent abortion, thrombocytopenia and neurological disorders Musculoskeletal manifestations and mucocutaneous symptoms occur frequently. Anaesthetic management of pregnant patients with SLE depends on the multisystem nature of the disease, the severity of the organ involvement and adverse effects of drugs used in treatment. Conclusion : Anaesthetic management of SLE requires thorough pre anaesthetic evaluation regarding organ involvement , disease activity and drug history . Pharmacological interactions between anaesthetic drugs and immunosuppressants taken into consideration

Incidence of rheumatic diseases during the COVID-19 pandemic in South Korea.

The recent coronavirus disease 2019 (COVID-19) pandemic has been associated with changes in the epidemiology of not only infectious diseases but also several non-infectious conditions. This study investigated changes in the recorded incidence of various rheumatic diseases during the COVID-19 pandemic. The number of patients for each disease from January 2016 to December 2020 was obtained from the Korean Health Insurance Review and Assessment Service database. We compared the incidence of nine rheumatic diseases (seropositive rheumatoid arthritis, systemic lupus erythematosus [SLE], idiopathic inflammatory myositis [IIM], ankylosing spondylitis [AS], systemic sclerosis, Sjögren's syndrome, Behçet's disease [BD], polymyalgia rheumatica, and gout) and hypertensive diseases to control for changes in healthcare utilisation before and after the COVID-19 outbreak. The disease incidence before and after the COVID-19 outbreak was compared using the autoregressive integrated moving average (ARIMA) and quasi- Poisson analyses. Compared with the predicted incidence in 2020 using the ARIMA model, the monthly incidence of SLE, BD, AS, and gout temporarily significantly decreased, whereas other rheumatic diseases and hypertensive diseases were within the 95% confidence interval (CI) of the predicted values in the first half of 2020. In age- and sex-adjusted quasi-Poisson regression analysis, the annual incidences of IIM (rate ratio [RR], 0.473; 95% CI, 0.307 to 0.697), SLE (RR, 0.845; 95% CI, 0.798 to 0.895), and BD (RR, 0.850; 95% CI, 0.796 to 0.906) were significantly decreased compared with those in the previous 4 years. The recorded annual incidence of some rheumatic diseases, including IIM, SLE, and BD, decreased during the COVID-19 pandemic.

Systemic lupus erythematosus incidence and prevalence in a large population-based study in northeastern Italy.

We aimed at estimating the incidence and prevalence of SLE in northeastern Italy over the period 2012-20. A retrospective population-based study was conducted in Veneto Region (4.9 million people) using the population registry, an administrative health database where all residents are recorded. Between 2012 and 2020, SLE prevalence was defined by a healthcare co-payment exemption for SLE (national registry code 028) or any hospital diagnosis of SLE (International Classification of Disease , Ninth Revision, Clinical Modification 710.0), whichever came first. Incident SLE was defined from 2013 to 2020 to exclude prevalent cases. Standardized incidence and prevalence rates were reported by age and sex. During the study period, we identified 4283 SLE patients (85% female), with 1092 incident cases. Across the study period, SLE standardized point prevalence increased from 63.5 (95% CI 61.2, 65.8) to 70.6 (95% CI 68.3, 73.0) per 100 000 residents, corresponding to an annual increment of 1.14% (P < 0.0001). The highest prevalence was observed in females aged 60-69 years. SLE incidence corresponded to 2.8 per 100 000 person-years (95% CI 2.6, 2.9), with an annual decline of 7.3% (P < 0.0001). Incidence was 5-fold higher in females (female-to-male incidence rate ratio: 5.00, 95% CI 4.25, 5.87; P < 0.0001), with a peak among women aged 30-39 years. At diagnosis, women were significantly younger (45 years, IQR 33-58) than men (52 years, IQR 38-64). Over the last decade, SLE prevalence has increased, while incidence has stably declined. In view of the introduction of new high-cost drugs, a clear definition of the epidemiology of SLE is crucial for all healthcare stakeholders.

Follow-up of clinical activity and accumulated organic damage in a cohort of patients with systemic lupus erythematosus from the Yucatán Peninsula, Mexico (1995–2016)

IntroductionSystemic lupus erythematosus (SLE) is an autoimmune with variable severity, common in Hispanic and African-American individuals. ObjectiveTo know the clinical activity and the accumulated damage, as well as the prevalence and incidence, in a dynamic cohort of patients with SLE from the Yucatan Peninsula (1995–2016). Patients and MethodsA cohort of 200 patients with SLE, medical service beneficiaries of the ISSSTE Regional Hospital of Mérida, Yucatán, was analysed for 22 years. Disease activity and accumulated damage were evaluated using the MEX-SLEDAI scale and the SLICC-ACR-DI, respectively, and its correlation with clinical and demographic variables. Results185 female and 15 male patients were analysed. Average accumulated damage and activity indices during follow-up were 4.63 and 1.10, respectively. The activity index was significantly lower in females compared to males (4.36 vs 7.43), and the accumulated damage did not present a difference by sex. The manifestations associated with greater activity were the mucocutaneous and articular ones, and the organs with the greatest accumulated damage were the musculoskeletal, neurological and gonadal. A relationship between the indices was found with the evolution time, remissions/reactivations, and persistent activity. Mortality was related to persistent activity due to systemic vascular complications and kidney and liver failure. The annual incidence and prevalence of SLE calculated was 2.86% and 48.43% in Yucatán Peninsula. ConclusionsThe patients presented persistent activity, with mild to moderate reactivations, and accumulated damage more aggressive in men. The clinical activity decreases and increases the accumulated damage at a longer evolution time, with less kidney disease and greater survival, which suggests a more benign course in the population of the Yucatan Peninsula.

Characteristics of childhood-onset systemic lupus erythematosus in pregnancy and its association with pregnancy outcomes: a retrospective comparative cohort study

BackgroundDisease situations are more aggressive in patients with childhood-onset systemic lupus erythematosus (cSLE) than in those with adult-onset SLE (aSLE). However, information on pregnant women with cSLE and its association with pregnancy outcomes is limited. This study aimed to compare pregnancies in patients with cSLE vs. aSLE, and further analyse the characteristics of cSLE in pregnant women and explore its association with adverse pregnancy outcomes.MethodsAltogether, data of 167 pregnancies from 150 women, including 22 pregnancies with cSLE and 145 pregnancies with aSLE, were retrospectively analysed. Characteristics and disease activity were compared between the cSLE and aSLE groups during pregnancy. Associations between cSLE and the risk of active SLE (SLEPDAI > 4), active lupus nephritis (LN), and adverse pregnancy outcomes were analysed using logistic regression.ResultsThe cSLE group had a higher incidence of active SLE (12/22 vs. 30/145, P = 0.001) and active LN (11/22 vs. 26/145, P = 0.001) than the aSLE group. In the multivariable analysis, cSLE was a risk factor for active SLE and active LN during pregnancy, with ORs of 4.742 (95%CI 1.678–13.405, P = 0.003) and 4.652 (95%CI 1.630–13.279, P = 0.004), respectively. No significant association between cSLE and the risk of composite adverse gestational outcomes was identified after sequentially adjusting pre-pregnancy characteristics and pregnancy factors (P > 0.05).ConclusionDisease activity of women with cSLE in pregnancy was more aggressive than that of women with aSLE, which was similar to the characteristics of non-pregnant women with SLE. cSLE might have indirect effects on the risk of adverse pregnancy outcomes through LN and active disease. Therefore, closely monitoring patients with cSLE during pregnancy is crucial.

Population-based incidence and time to classification of systemic lupus erythematosus by three different classification criteria: a Lupus Midwest Network (LUMEN) study.

To estimate the incidence and time-to-classification of SLE by the 1997 ACR (ACR97) criteria, the SLICC criteria, and the European Alliance of Associations for Rheumatology/ACR (EULAR/ACR) criteria. We identified all incident SLE cases from 2000-2018 in the well-defined Olmsted County population. Clinical data included in the ACR97, SLICC and EULAR/ACR criteria were manually abstracted from medical records. All incident cases met at least one of the three classification criteria. Time-to-classification was estimated from the first documented lupus-attributable disease manifestation to the time of criteria fulfilment by each of the three definitions. Annual incidence rates were age or age/sex adjusted to the 2000 US population. Of 139 incident cases there were 126 cases by the EULAR/ACR criteria, corresponding to an age/sex-adjusted incidence of 4.5 per 100000 population (95% CI: 3.7, 5.2). The age/sex-incidence was higher than that of the SLICC criteria (113 cases; 4.0 per 100000 [95% CI: 3.3, 4.7], P = 0.020) and the ACR97 (92 cases; 3.3 per 100000 [95% CI: 2.6, 3.9], P < 0.001). The median time from first disease manifestation to criteria fulfilment was shorter for the EULAR/ACR criteria (29.4 months) than the ACR97 criteria (47.0 months, P < 0.001) and similar to the SLICC criteria (30.6 months, P = 0.83). The incidence of SLE was higher by the EULAR/ACR criteria compared with the ACR97 and the SLICC criteria, and the EULAR/ACR criteria classified patients earlier that the ACR97 criteria but similar to the SLICC criteria.

PSY10 Global and Regional Prevalence and Incidence of LUPUS in Low and-Middle Income Countries: A Systematic Review and Meta-Analysis

The aim of this study was to systematically summarise the prevalence and incidence of SLE in low and-middle income countries (LMICs). A systematic search of MEDLINE, CINHAL, Web of Science and Global Index Medicus was carried out using keyword search terms for SLE combined with prevalence, incidence and epidemiology. The search was to identify papers addressing SLE in LMICs and published up to September 1, 2020. The prevalence and incidence of SLE were pooled through random effect model. Pooled estimates were expressed with 95% confidence intervals. Out of 1757 papers, 16 studies were included. The mean age at diagnosis ranged from 25.5 to 45.8 years. The included studies were conducted in Argentina (n = 2), Brazil (n = 3), China (n = 2), Colombia, Greece, India, Malaysia, Pakistan, Venezuela, Zimbabwe and French Guiana. The SLE prevalence and incidence varied from 3.2 to 159 per 100,000 and 1.4 to 8.7 per 100,000 population, respectively. In the random effects meta-analysis (n = 8), the pooled prevalence of SLE was 120 (95% confidence interval [CI]:100 – 200) per 100,000. Meta-analysis of data from six incidence studies revealed an incidence of 4 cases per year (95% CI: 2 – 6) per 100,000. According WHO regions, the pooled prevalence of American and Western Pacific regions was 300 (95% CI -200 – 900) and 36 (95% CI 35 – 37) per 100,000, respectively. The pooled incidence of the American region was 10 (95% 0 – 14) per 100,000 population. Our findings provide a comprehensive and up-to-date summary of the prevalence and incidence of SLE in LMICs. As SLE is an important threat to the population, health professionals and policymakers needs to put in place appropriate programmes to reduce the burden of SLE in LMICs.

A Case of Papulonodular Mucinosis in a Patient With Systemic Lupus Erythematosus

Papulonodular mucinosis is a distinctive lupus erythematosus cutaneous variant that generally occurs in patients with systemic lupus erythematosus. Despite a higher incidence of SLE in women, lupus cutaneous mucinosis occurs more frequently in men. Typically, papulonodular mucinosis appears as asymptomatic, flesh-colored papules and nodules with a propensity for the trunk and upper extremities. Herein, we report a case of papulonodular mucinosis associated with systemic lupus erythematosus in a middle-aged woman. On presentation, patient had multiple flesh-colored papules coalescing into plaques on the trunk and upper extremities. Histological findings of the lesions demonstrated a pale dermis secondary to profuse mucin deposition. Immunofluorescent staining revealed a 'lupus band' with granular deposition of immunoglobulins/complement proteins at the dermal-epidermal junction.

Serologic features of cohorts with variable genetic risk for systemic lupus erythematosus

BackgroundSystemic lupus erythematosus (SLE) is an autoimmune disease with genetic, hormonal, and environmental influences. In Western Europe and North America, individuals of West African descent have a 3–4 fold greater incidence of SLE than Caucasians. Paradoxically, West Africans in sub-Saharan Africa appear to have a low incidence of SLE, and some studies suggest a milder disease with less nephritis. In this study, we analyzed sera from African American female SLE patients and four other cohorts, one with SLE and others with varying degrees of risk for SLE in order to identify serologic factors that might correlate with risk of or protection against SLE.MethodsOur cohorts included West African women with previous malaria infection assumed to be protected from development of SLE, clinically unaffected sisters of SLE patients with high risk of developing SLE, healthy African American women with intermediate risk, healthy Caucasian women with low risk of developing SLE, and women with a diagnosis of SLE. We developed a lupus risk index (LRI) based on titers of IgM and IgG anti-double stranded DNA antibodies and levels of C1q.ResultsThe risk index was highest in SLE patients; second highest in unaffected sisters of SLE patients; third highest in healthy African-American women and lowest in healthy Caucasian women and malaria-exposed West African women.ConclusionThis risk index may be useful in early interventions to prevent SLE. In addition, it suggests new therapeutic approaches for the treatment of SLE.

Loss of B cell Ezrin in lupus-prone Lyn-deficient mice downregulates B cell activation and autoimmune pathology

Abstract Systemic lupus erythematosus (SLE) is an aggressive autoimmune disorder that afflicts 1.5 million Americans. Human GWAS studies have reported a strong association between the Src family tyrosine kinase Lyn and incidence of SLE, and B cells from SLE patients exhibit decreased Lyn expression. Genetic deletion of Lyn in mice results in loss of peripheral B cell tolerance and development of human SLE-like autoimmunity, characterized by hyperactive B cell antigen receptor (BCR) signaling, autoantibody production and glomerulonephritis. However, the molecular regulators of B cell pathogenesis in Lyn-deficient mice have not been fully characterized. We have previously shown that the membrane-cytoskeleton linker protein ezrin regulates multiple aspects of B cell function through its dynamic switching between phosphorylated and dephosphorylated states. We observed that ezrin is hyperphosphorylated in Lyn-deficient B cells, suggesting that it may impact B cell activation in this context. To test this hypothesis, we generated conditional deletion of ezrin in the B cells of Lyn-deficient mice (hereafter referred to as DKO mice). Compared to Lyn-deficient mice, the DKO mice exhibited a significant decrease in splenomegaly and reduction in autoantibodies against a variety of autoantigens. Glomerulonephritis and deposition of IgG and complement factor C3 in the kidneys of DKO mice were also markedly reduced. The DKO B cells were hyporesponsive to BCR stimulation and displayed a calcium mobilization pattern reminiscent of that observed in anergic B cells. Our data demonstrate that ezrin regulates B cell hyperactivation and associated pathology in the absence of Lyn, and suggest that ezrin may be a novel molecular target in SLE.

Cutaneous manifestations of systemic lupus erythematosus

&lt;p class="abstract"&gt;&lt;strong&gt;Background:&lt;/strong&gt; SLE is a systemic autoimmune disorder associated with polyclonal activation and interplay of genetic, environmental and hormonal events. The aim of the study was to study the incidence of mucocutaneous spectrum, precipitating factors, HPE, immunofluorescence and lab data.&lt;/p&gt;&lt;p class="abstract"&gt;&lt;strong&gt;Methods:&lt;/strong&gt; Prospective cohort study. 100 SLE patients attending MMC from August 2007 to September 2009 are included in study. Detailed history, investigations and 2 years follow up were done.&lt;strong&gt;&lt;/strong&gt;&lt;/p&gt;&lt;p class="abstract"&gt;&lt;strong&gt;Results:&lt;/strong&gt; ACLE lesions were seen in 50% patients. Sunlight was the most common precipitating factors [55%]. Mucocutaneous lesions are seen in 45%. Fever is the most common systemic symptom in 45.45%. Photosensitivity is seen in 98% of patients. DLE lesions are seen in 83.66% of patients.98.5% of histopathology correlates with classical SLE.&lt;/p&gt;&lt;p class="abstract"&gt;&lt;strong&gt;Conclusions:&lt;/strong&gt; Most of the results correlate with previous studies with few rare observations. Incidence of SLE is less compared to the Western part of world which may be due to the genetic and environmental factors. Photosensitivity being the most common symptom and sunlight being the most common exaggerating factor in our study may be due to the unprotected environmental exposure. Age, sex, ethnic factor with genetic predisposition are the main modifying factors of the various presentation of the disorder.&lt;/p&gt;

The worldwide incidence and prevalence of systemic lupus erythematosus: a systematic review of epidemiological studies.

The aim was to review the worldwide incidence and prevalence of SLE and variation with age, sex, ethnicity and time. A systematic search of MEDLINE and EMBASE search engines was carried out using Medical Subject Headings and keyword search terms for Systemic Lupus Erythematosus combined with incidence, prevalence and epidemiology in August 2013 and updated in September 2016. Author, journal, year of publication, country, region, case-finding method, study period, number of incident or prevalent cases, incidence (per 100 000 person-years) or prevalence (per 100 000 persons) and age, sex or ethnic group-specific incidence or prevalence were collected. The highest estimates of incidence and prevalence of SLE were in North America [23.2/100 000 person-years (95% CI: 23.4, 24.0) and 241/100 000 people (95% CI: 130, 352), respectively]. The lowest incidences of SLE were reported in Africa and Ukraine (0.3/100 000 person-years), and the lowest prevalence was in Northern Australia (0 cases in a sample of 847 people). Women were more frequently affected than men for every age and ethnic group. Incidence peaked in middle adulthood and occurred later for men. People of Black ethnicity had the highest incidence and prevalence of SLE, whereas those with White ethnicity had the lowest incidence and prevalence. There appeared to be an increasing trend of SLE prevalence with time. There are worldwide differences in the incidence and prevalence of SLE that vary with sex, age, ethnicity and time. Further study of genetic and environmental risk factors may explain the reasons for these differences. More epidemiological studies in Africa are warranted.

How to Identify High-Risk APS Patients: Clinical Utility and Predictive Values of Validated Scores.

Antiphospholipid syndrome (APS) is a clinical disorder characterised by thrombosis and/or pregnancy morbidity in the persistence of antiphospholipid (aPL) antibodies that are pathogenic and have pro-coagulant activities. Thrombosis in APS tends to recur and require prophylaxis; however, the stereotypical treatment for APS patients is inadequate and stratification of the thrombotic risks is important as aPL are prevalently observed in various diseases or elderly population. It is previously known that the multiple positive aPL or high titre aPL correlate to thrombotic events. To progress the stratification of thrombotic risks in APS patients and to quantitatively analyse those risks, antiphospholipid score (aPL-S) and the Global Anti-phospholipid Syndrome Score (GAPSS) were defined. These scores were raised from the large patient cohort data and either aPL profile classified in detail (aPL-S) or simplified aPL profile with classical thrombotic risk factors (GAPSS) was put into a scoring system. Both the aPL-S and GAPSS have shown a degree of accuracy in identifying high-risk APS patients, especially those at a high risk of thrombosis. However, there are several areas requiring improvement, or at least that clinicians should be aware of, before these instruments are applied in clinical practice. One such issue is standardisation of the aPL tests, including general testing of phosphatidylserine-dependent antiprothrombin antibodies (aPS/PT). Additionally, clinicians may need to be aware of the patient's medical history, particularly with respect to the incidence of SLE, which influences the cutoff value for identifying high-risk patients.