Features Of Systemic Lupus Erythematosus Research Articles

Features, Treatment, and Outcomes of Macrophage Activation Syndrome in Childhood-Onset Systemic Lupus Erythematosus.

To describe the features and treatment of macrophage activation syndrome (MAS) in a single-center cohort of patients with childhood-onset systemic lupus erythematosus (SLE), and to compare childhood-onset SLE manifestations and outcomes between those with and those without MAS. We included all patients with childhood-onset SLE followed up at The Hospital for Sick Children from 2002 to 2012, and identified those also diagnosed as having MAS. Demographic, clinical, and laboratory features of MAS and SLE, medication use, hospital and pediatric intensive care unit (PICU) admissions, as well as damage indices and mortality data were extracted from the Lupus database. Student's t-tests and Fisher's exact tests were used to compare continuous and categorical variables, respectively. We calculated incidence rate ratios of hospital and PICU admissions comparing patients with and those without MAS, using Poisson models. Kaplan-Meier survival analysis was used to examine the time to disease damage accrual. Of the 403 patients with childhood-onset SLE, 38 (9%) had MAS. The majority (68%) had concomitant MAS and SLE diagnoses. Fever was the most common MAS clinical feature. The frequency of renal and central nervous system disease, hospital admissions, the average daily dose of steroids, and time to disease damage were similar between those with and those without MAS. We observed a higher mortality rate among those with MAS (5%) than those without MAS (0.2%) (P = 0.02). MAS was most likely to develop concomitantly with childhood-onset SLE diagnosis. The majority of the MAS patients were successfully treated with corticosteroids with no MAS relapses. Although the numbers were small, there was a higher risk of death associated with MAS compared to SLE without MAS.

Systemic lupus erythematosus presenting to haematology with pancytopenia and features of macrophage activation syndrome

This bone marrow biopsy (figure 1) shows haemophagocytosis consistent with macrophage activation syndrome (MAS) secondary to previously undiagnosed systemic lupus erythematosus (SLE). Figure 1 Bone marrow biopsy with abundant cell-bound...

Characteristics of systemic lupus erythematosus in a sample of the Egyptian population: a retrospective cohort of 1109 patients from a single center.

Introduction Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease that can vary among different ethnic and racial groups. Objective The objective of this paper is to study the prevalence of various manifestations of SLE in a sample of the Egyptian population. Patients and methods Information in this study was derived from the medical records of SLE patients who sought medical advice at a private clinic in Cairo from January 1980 to June 2016. Results This study included 1109 SLE patients, of whom 114 (10.3%) were males and 995 were females (89.7%). Mean age of onset was 25.89 ± 10.81 years, while the median of disease duration from the onset of the disease till the last recorded visit was 26 months. The most common cumulative manifestations were arthritis (76.7%), malar rash (48.5%), leukopenia (45.7%), and photosensitivity (45.6%). A total of 33.1% of the patients had nephritis, and neuropsychiatric lupus was present in 6.4% of the patients. Secondary antiphospholipid syndrome was present in 11.5% of the patients. Antinuclear antibody and anti-double-stranded deoxyribonucleic acid were present in 1060/1094 (96.9%) and 842/1062 (79.3%) of the patients, respectively. Antiphospholipid antibodies were present in 266/636 (41.8%) of the patients, anti-Smith in 54/240 (22.5%), anti-SSA/Ro in 61/229 (20.4%), and anti-SSB/La in 32/277 (11.6%) of the patients. Male patients had a statistically higher prevalence of nephritis ( p = 0.01), whereas arthritis and alopecia were statistically higher in females ( p = 0.012 and p = 0.006, respectively). Patients with juvenile onset had a statistically higher prevalence of nephritis and seizures ( p < 0.001 and p = 0.012, respectively). Conclusions Arthritis and malar rash represented the most common clinical manifestations. Male and juvenile-onset patients had a predilection toward a more severe disease. These results are in agreement with many studies conducted in the Middle East and worldwide. On the other hand, major organ involvement was exceptionally low, which is contradictory to several reports from the Middle East and across the globe.

Serum complement factor I is associated with disease activity of systemic lupus erythematosus.

Although aberrant complement activation is involved in the pathogenesis of systemic lupus erythematosus (SLE), the role of complement regulatory proteins in disease activity of SLE remains limited. We enrolled the pediatric-onset SLE patients from our cohort study over 10 years. The clinical and laboratory data including SLEDAI disease activity score, and serum complement factor H (CFH), CFI, CD46, C5a, and C5b-9 in the active and remission phases were determined. Glomerular C5b-9 deposition as a complement activity marker was also examined. Forty patients (35 female and 5 male, aged 13.9 ± 3.8 years) met the criteria of investigation were assessed. Fever and kidney were the most common symptom and organ involved, respectively. Mean SLEDAI in the active and remission phases were 12.6 vs 1.7, respectively. All patients exhibited lower serum C3, C4, CFH and CFI and higher serum anti-dsDNA and CD46 in the active pahse. There was a significant difference in serum CFH, CFI and CD46 between active and remissive phases. Serum CFI but not CFH and CD46 level was negatively correlated with SLEDAI score in active phase. Compared to classical activity markers, serum CFI was superior to C4 and anti-dsDNA in reflecting disease activity and also significantly correlated with white blood count and hemoglobin. Glomerular C5b-9 depositions were detected in patients with nephritis during active phase but not in disease controls. Serum CFI level may not only be a promising biomarker for disease activity of SLE, but also reflects the hematological features of SLE.

Cellular Microvesicles in the Blood of Patients with Systemic Lupus Erythematosus

Systemic lupus erythematosus (SLE) is an autoimmune disease with a complex and largely unclear pathogenesis. Cellular phospholipid microvesicles released upon activation and/or death of a cell have been proposed to play a role in inflammatory autoimmune pathologies, including SLE. Here, circulating microvesicles of various cellular origins were marked with fluorescently labeled cell-specific antibodies and enumerated by flow cytometry in platelet-free plasma obtained from the heparinized blood of 29 SLE patients and 19 normal subjects. Significantly higher concentrations of endothelial-, monocyte-, and erythrocyte-derived microvesicles were found in the SLE patients compared to normal subjects with prevalence of microvesicles originating from endothelial cells. No significant difference was found for platelet-derived microvesicles. A correlation analysis of microvesicle counts with laboratory parameters and clinical features of SLE suggest differential implications of various cell-derived microvesicles in the pathogenesis of SLE. These data suggest that SLE is associated with functional alterations of endotheliocytes, monocytes, and erythrocytes followed by enhanced release of microvesicles that may contribute to inflammation and hypercoagulability.

Long-term efficacy and safety of antitumour necrosis factor alpha treatment in rhupus: an open-label study of 15 patients

BackgroundThe efficacy of antitumour necrosis factor alpha (anti-TNF-α) treatment is well recognised in rheumatoid arthritis (RA) but remains controversial in systemic lupus erythematosus (SLE). Therefore, the role of anti-TNF-α treatment...

A Novel Human Systemic Lupus Erythematosus Model in Humanised Mice

Mouse models have contributed to the bulk of knowledge on Systemic Lupus Erythematosus (SLE). Nevertheless, substantial differences exist between human and mouse immune system. We aimed to establish and characterise a SLE model mediated by human immune system. Injection of pristane into immunodeficient mice reconstituted with human immune system (humanised mice) recapitulated key SLE features, including: production of human anti-nuclear autoantibodies, lupus nephritis, and pulmonary serositis. There was a reduction in the number of human lymphocytes in peripheral blood, resembling lymphopenia in SLE patients. Concurrently, B cells and T cells were systemically hyperactivated, with a relative expansion of CD27+ and CD27−IgD− memory B cells, increased number of plasmablasts/plasma cells, and accumulation of effector memory T cells. There was also an increased production of human pro-inflammatory cytokines, including: IFN-γ, IL-8, IL-18, MCP-1, and IL-6, suggesting their role in SLE pathogenesis. Increased expression of type I IFN signature genes was also found in human hepatocytes. Altogether, we showed an SLE model that was mediated by human immune system, and which recapitulated key clinical and immunological SLE features. The advancements of humanised mice SLE model would provide an in vivo platform to facilitate translational studies and pre-clinical evaluations of human-specific mechanisms and immunotherapies.

Earlier Development of Limb Ulcers, Digital Bone Infarction and Pulmonary Hypertension in a Patient with SLE- A Rare Case Report

Systemic lupus erythematosus (SLE) is a multiorgan autoimmune disease in which skin is involved in up to 85% of cases. Lower extremity ulcers are an infrequent but disabling complication of SLE. Critical peripheral ischemia (CPI) is also an uncommon but potentially devastating feature of SLE. We reported a case o f SLE who presented with multiple ulcers along with digital infarcts of upper and lower limbs. She had features of digital bone infarct of left hand and right foot. She also had features of pulmonary interstitial involvement with pulmonary hypertension. All of these features were found within three months of disease onset which is very rare. She was treated with intravenous Methylprednisolone followed by oral Prednisolone, Hydroxychloroquinine, Azathioprine, Diuretic, Bosentan, Aspirin and Nifedipine in combinaton. Later warfarin was added after one month. She showed significant improvement after three months of treatment.J Bangladesh Coll Phys Surg 2017; 35(3): 150-154

Exploring the mechanism of Jieduquyuziyin prescription on systemic lupus erythematosus by GC-MS-based urine metabolomics.

A urine metabolomics method based on gas chromatography-mass spectrometry was developed in order to investigate the metabolite characteristics of systemic lupus erythematosus (SLE) and the therapeutic effects of Jieduquyuziyin prescription. The urinary metabolic profiles in urine specimens of the SLE model mice (MRL/lpr) group, prednisone acetate-treated SLE mouse group, Jieduquyuziyin prescription-treated SLE mouse group, and control group (C57BL/6 J) after the administration were analyzed by gas chromatography-mass spectrometry. These metabolic profiles were then processed by multivariate analysis, in particular Mass Profiler Professional, SIMCA-P and partial least-squares discriminant analysis. According to the partial least-squares discriminant analysis results, the SLE model group and the control group were obviously separated, indicating that the incidence of SLE had a greater impact on the metabolic network, and the SLE model group had significant difference compared with the control group in urine metabolites. Eleven differential metabolites were identified to be related to SLE, and the results of differential metabolite identification showed that the metabolites were mainly related to energy metabolism and amino acid metabolism pathway. These results can provide an experimental basis for further exploring the mechanism of traditional Chinese medicine in the treatment of SLE.

Cardiac tamponade in systemic lupus erythematosus.

Cardiac tamponade is a rare but life-threatening complication of systemic lupus erythematosus (SLE). To describe incidence, risk factors and treatment of cardiac tamponade in a large cohort of Indian patients with SLE. This retrospective study was conducted at the Department of Rheumatology, IPGMER, Kolkata, India from May 2014 to December 2016 on admitted patients with SLE. Lupus-related serositis was diagnosed after excluding other causes, such as infection, malignancy or heart failure. Of 409 patients with SLE, pericarditis was diagnosed in 25.4% (104/409) and cardiac tamponade in 5.9% (24/409). Tamponade was the presenting feature of SLE in 50% (12/24). Tamponade occurred in 77.8% (14/18) of large effusions and in 11.63% (10/86) of small-to-moderate effusions. The commonest autoantibody in serum and pericardial fluid was anti-nucleosme antibody. Large pericardial effusion (>20 mm) (Odd's ratio (OR): 93.2, 95% confidence interval (CI): 11.1-782.5, P < 0.001) predicted tamponade. In the subset of patients with small-to-moderate sized pericardial effusion, tamponade was associated with pleuritis (OR: 44.5, 95% CI: 1.6-1243, P = 0.025), anti-nucleosome antibody (OR: 42.9, 95% CI: 1.6-1176, P = 0.026) and size of pericardial effusion (OR: 1.36, 95% CI: 1.04-1.76, P = 0.025). Repeated pericardiocentesis was required in 3 patients and one needed surgical intervention. Immunosuppressives used were: prednisolone with monthly intravenous cyclophosphamide (in 33.33%) and intravenous methylprednisolone with monthly cyclophosphamide (in 50%). Pleuritis, anti-nucleosome antibody and size of pericardial effusion predicted development of tamponade. High dose immunosuppression (methylprednisolone and IV cyclophosphamide) alleviated need for surgery in majority.

Temperament and character in patients with systemic lupus erythematosus

BackgroundIn addition to identified neuropsychiatric characteristic of systemic lupus erythematosus (SLE), changes in personality seem to occur in patients with SLE. Even in absence of an axis I psychiatric diagnosis, personality variations play important role in general wellbeing of these patients. This study investigated personality features in patients with SLE using Temperament and Character Inventory (TCI). MethodIn this case-control study personality features of 59 patients with confirmed diagnosis of SLE were evaluated using Persian version of TCI-125 questionnaire. Collected data from patients with SLE were statistically compared with normative data for Iranian population. ResultsAmong four subscales of temperament, reward dependence (RD) and harm-avoidance (HA) were significantly lower than general population. Self-directedness (SD) character dimension was significantly lower in SLE patients compared to normative data. No significant difference was noted in novelty-seeking (NS) and persistence (PS) temperament scales and cooperativeness (CO) and self-transcendence (ST) character scales. ConclusionPersonality changes in SLE is characterized by higher HA and RD along with low SD. These features are associated with higher anxiety, social withdrawal and lower resourcefulness and purposefulness.

Clinical analysis of late-onset systemic lupus erythematosus in Xinjiang

Objective To assess the clinical characteristics of systemic lupus erythematosus (SLE) in the elderly in Xinjiang. Methods As a retrospective study, clinical date and laboratory test results were collected from 87 elderly SLE patients and 222 non-elderly SLE patients (age <50 years)hospitalized in the People's Hospital of the Xinjiang Uygur Autonomous Region from January 2006 to July 2016. Results The female to maleratio was 5.7∶1.0 in the older-onset SLE group and the female to male ratio was 11.3∶1.0 in the younger-onset SLE group.Of the 13 patients with predisposing factors in the older-onset SLE group(14.9%), 11 had infection (12.6%), whereas the 27 patients with predisposing factors in the younger-onset SLE group (12.2%)had pregnancy, abortion or insolation, in addition to infection.Arthritis(63.2%), weakness(40.2%), malar rashes(36.8%), anorexia(26.4%), and shortness of breath(26.4%) were common clinical manifestations in the older-onset SLE group.The incidence of anorexia in the older-onset SLE group was higher than that in the younger-onset SLE group(P<0.01), while the incidence of trichomadesis was lower in the older-onset SLE group than that in the younger-onset SLE group(P<0.01); Aminotransferase elevation, creatinine elevation and thrombocytopenia were more common in the older-onset SLE group than in the younger SLE group (P<0.05). Lower rates of positive anti-Smanti body and anti-Acl antibody were found in the older-onset SLE group, compared with the younger-onset SLE group (P<0.05); Han older-onset SLE patients showed higher rates of oral ulcers than older-onset SLE patients of Uygur, Kazak or other ethnic minorities (P<0.05). There were no significant differences between Han and Uygur/Kazak patients in laboratory test results. Conclusions The proportion of male SLE patients in the elderly is higher than that in non-elderly SLE patients in Xinjiang.Also, elderly SLE patients are prone to oral ulcers but often do not show typical early clinical symptoms and have low levels of specific antibodies.Therefore, clinicians should pay particular attention to older-onset SLE patients in order to reduce the rates of misdiagnosis and missed diagnosis. Key words: Lupus erythematosus; systemic

A retrospective study of joint infections in patients with systemic lupus erythematosus

The aim of this study was to analyze the clinical characteristics of systemic lupus erythematosus (SLE) patients with joint infections. We retrospectively reviewed the medical records of 11,734 SLE patients admitted to Peking Union Medical College Hospital (PUMCH) from January 1990 to December 2016. Twenty patients who developed joint infections were identified. Subjects without joint infections (designated as control patients) were selected from the pool of SLE patients using a 1:4 systematic sampling method. The median disease duration from SLE onset to joint infection was 23months (range 4 to 156months). The symptoms of patients with joint infections manifested as joint pain (all cases), swelling (14 cases), and fever (15 cases). All patients had oligo-arthritis, and the knee was the joint most commonly affected joint. There were 7 patients in the Salmonella group and 5 in the Staphylococcus aureus group. One patient was infected with Streptococcus, and 7 patients were infected with Mycobacterium. SLE patients with and without joint infections demonstrated significant differences (P<0.05) regarding the following symptoms: pre-existing arthritis (65.0 vs 33.8%), gastrointestinal involvement (5.0 vs 26.3%), cardiac damage (5.0 vs 31.3%), elevated C-reactive protein (CRP) (80.0 vs 22.5%), and elevated SLE Disease Activity Index (SLEDAI) score (≥5) (30.0 vs 77.5%). When an SLE patient presents with pre-existing arthritis and suddenly develops asymmetric oligo- or large-joint swelling and pain with elevated CRP levels and low disease activity, joint infections should be considered. Early treatment could protect the joint and improve functional outcomes.

Periodontal disease in Chinese patients with systemic lupus erythematosus.

Disease of systemic lupus erythematosus (SLE) and periodontal disease (PD) shares the common multiple characteristics. The aims of the present study were to evaluate the prevalence and severity of periodontal disease in Chinese SLE patients and to determine the association between SLE features and periodontal parameters. A cross-sectional study of 108 SLE patients together with 108 age- and sex-matched healthy controls was made. Periodontal status was conducted by two dentists independently. Sociodemographic characteristics, lifestyle factors, medication use, and clinical parameters were also assessed. The periodontal status was significantly worse in SLE patients compared to controls. In univariate logistic regression, SLE had a significant 2.78-fold [95% confidence interval (CI) 1.60-4.82] increase in odds of periodontitis compared to healthy controls. Adjusted for potential risk factors, patients with SLE had 13.98-fold (95% CI 5.10-38.33) increased odds against controls. In multiple linear regression model, the independent variable negatively and significantly associated with gingival index was education (P=0.005); conversely, disease activity (P<0.001) and plaque index (P=0.002) were positively associated; Age was the only variable independently associated with periodontitis of SLE in multivariate logistic regression (OR 1.348; 95% CI: 1.183-1.536, P<0.001). Chinese SLE patients were likely to suffer from higher odds of PD. These findings confirmed the importance of early interventions in combination with medical therapy. It is necessary for a close collaboration between dentists and clinicians when treating those patients.

Macrophage Activation Syndrome as the Initial Manifestation of Systemic Lupus Erythematosus in a 7-Year-Old Girl – A Case Report.

Macrophage activation syndrome (MAS) is a potentially fatal complication of several chronic rheumatic diseases. Although it occurs most commonly with systemic onset juvenile idiopathic arthritis (SoJIA), it may also occur in association with systemic lupus erythematosus (SLE), Kawasaki disease, and adult onset Still’s disease. It is usually triggered by infections or due to medication modification. Here we report a 7-year-old girl who presented simultaneously with features of MAS and SLE and responded with pulse methylprednisolone followed by oral prednisolone and pulse cyclophosphamide. Keywords: Macrophage Activation Syndrome (MAS); Systemic Lupus Erythematosus (SLE); Child.

Anti-nucleosome antibodies in systemic lupus erythematosus patients: Relation to anti-double stranded deoxyribonucleic acid and disease activity

Aim of the workTo measure the level of anti-nucleosome (anti-NCS) antibodies in systemic lupus erythematosus (SLE) patients and to evaluate their relation with anti-double stranded deoxyribonucleic acid (anti-dsDNA) antibodies and SLE disease activity. Patients and methods66 Egyptian SLE patients were investigated for the detection of anti-NCS antibodies and anti-dsDNA antibodies. Disease activity was assessed using the SLE disease activity index (SLEDAI) and the European consensus lupus activity measurement (ECLAM). ResultsThe median age of the patients was 25.5years (12–48years) and disease duration 3years (1month to 26years). anti-NCS antibody was found in 48 (72.7%) patients. Non-significant difference was found between both those positive or negative anti-NCS antibodies regarding the clinical features apart from fever (p=0.019). Lupus nephritis was present in 35/48(72.9%) of those with positive and in 11/18 (61.1%) of those with negative anti-NCS (p=0.35) A significant correlation was found between anti-NCS antibodies with SLEDAI (r=0.36, p=0.003) and ECLAM (r=0.29, p=0.019). No significant relation was found between anti-NCS antibodies and clinical features of SLE, apart from fatigue (r=0.3, p=0.015). A significant correlation with hypocomplementemia (C3 r=−0.37, p=0.002 and C4 r=−0.32, p=0.018) and anaemia (r=−0.32, p=0.009). anti-dsDNA antibodies were detected in 35(53%) SLE patients; 70.8% of those with positive and 5.6% of those with negative anti-NCS antibodies. ConclusionAnti-NCS antibodies could play a role in the pathogenesis of SLE and is related to disease activity. Its association with anti-dsDNA antibodies and its presence in those with negative anti-ds DNA may aid in the diagnosis of SLE.

Modern clinical and epidemiological characteristic of systemic lupus erythematosus based on the data of the territorial registry

Systemic lupus erythematosus (SLE) is a severe rheumatic disease characterized by polysymptomatic clinical picture. At the present stage, there are no updated epidemiological data due to the low prevalence of the disease. The aim of the study was to examine the current clinical and epidemiological characteristics of patients with systemic lupus erythematosus based on the information contained in the territorial register, analysis of occurrence and symptoms at the early stage of the disease. This study demonstrated the epidemiological and clinical characteristics of SLE from the analysis of 107 cases during the period from 2011 to 2013 and retrospective analysis of the cases for 1980-2013. The epidemiological situation was evaluated based on extensive and intensive indicators using statistical software license. The current SLE prevalence was estimated at 5,59 per 100 000 population in 2013, the incidence between 1994 and 2003 at 0,29 per 100 000 population and between 2004 and 2013 at 0,49, with the peak in 2010 up to 1,35 per 100 000 population. The average absolute growth and growth rate of SLE in the first decade was 0,05% and 0,24%, in the second decade 0,001% and 0,006% respectively, with the female to male ratio being 9:1, mean age of the patients 37,62±11,65 years), and ethnic composition of 87 Slavs and 15 Crimean Tatars. The most common symptoms at the early (polyarthritis, fever, dermatitis) and advanced (polyarthritis, Raynaud's syndrome, carditis, myalgia) stages differed from those specified by American College of Rheumatology (1997). The difference between early and late symptoms of SLE was documented . Based on the data obtained, the division of the disease into clinical subtypes (phenotypes) is proposed.

Clinical picture of lupus nephritis in patients with systemic lupus erythematosus (SLE): Results of a large survey

Systemic lupus erythematosus (SLE) is a chronic multisystem disorder. Lupus nephritis (LN) is a common serious complication of SLE. LN needs prolonged care and complex therapeutic modalities. This study assessed the characteristics of Persian SLE patients with LN (LN subgroup) and an SLE subpopulation without LN (non-LN subgroup). Furthermore, the association of LN with extrarenal manifestations of SLE was studied. This study assessed 2355 SLE patients from the electronic database of the Rheumatology Research Center (RRC), Tehran University of Medical Sciences (TUMS). The clinical and laboratory data of enrolled patients was retrieved. The chi-square test was used to compare extrarenal manifestations of the LN and non-LN subgroups. Odds ratios (OR) were used to present the strength of associations. The LN subgroup included 1604 cases (68.1%) with a mean age at SLE onset of 24.6±12.5 years and a female-to-male ratio of 8.7/1. Class IV nephritis was the most common type of LN (53.1%). The comparison of extrarenal manifestations revealed statistically significant differences between LN and non-LN subgroups. Major organ involvement including cardiopulmonary, hematologic, musculoskeletal and neuropsychiatric features was significantly more common in LN patients. On the contrary, discoid rash was significantly more common in the non-LN subgroup. This study revealed that LN is positively associated with musculoskeletal, mucocutaneous, and neuropsychiatric features of SLE.

Sirtuin 1 (Sirt1) Overexpression in BaF3 Cells Contributes to Cell Proliferation Promotion, Apoptosis Resistance and Pro-Inflammatory Cytokine Production.

BackgroundB lymphocyte hyperactivity is a main characteristic of systemic lupus erythematosus (SLE), and B lymphocytes play a prominent pathogenic role in the development and progression of SLE. The aim of this study was to investigate the role of Sirtuin 1 (Sirt1) in B lymphocytes.Material/MethodsMouse B lymphocytes BaF3 was transfected with Sirt1 vector or shRNA against Sirt1. Then the transfected cells viability and apoptosis were respectively determined by MTT assay and flow cytometry. In addition, the mRNA levels of three pro-inflammatory cytokines and p53 were detected by RT-PCR. Furthermore, the expression levels of nuclear factor-kappa B (NF-κB) pathway proteins were measured by Western blot.ResultsOverexpression of Sirt1 significantly increased cell proliferation (p<0.05 or p<0.01) and significantly suppressed apoptosis (p<0.05). The mRNA level expressions of interleukin 1 (IL-1), IL-6, and tumor necrosis factor-α (TNF-α) were significantly upregulated (p<0.05 or p<0.01), whereas p53 was significantly downregulated (p<0.05) by Sirt1 overexpression. In addition, the inhibitory subunit of NF-κB (IκBα) and p65 were significantly activated and phosphorylated (p<0.01 or p<0.001), and B-Cell CLL/Lymphoma 3 (Bcl-3) was significantly upregulated (p<0.05) by Sirt1 overexpression.ConclusionsThese results suggested that Sirt1 overexpression could promote BaF3 cell proliferation, inhibit apoptosis, and upregulate pro-inflammatory cytokines. The NF-κB pathway might be involved in these effects of Sirt1 on BaF3 cells, and Sirt1 might be a potential risk factor of SLE.

Expression and its promoter methylation of chemokine CXC ligand 14 in peripheral blood mononuclear cells from patients with lupus

To analyze the expression and its promoter methylation of chemokine CXC ligand 14 (CXCL14) in peripheral blood mononuclear cells (PBMCs) from patients with systemic lupus erythematosus (SLE). The RNAs of PBMCs from 28 SLE patients and 20 healthy controls were isolated and reversely transcribed into cDNAs. Using GAPDH as the internal reference, the levels of CXCL14 ex-pression were detected by real-time polymerase chain reaction (PCR). The correlation between CXCL14 expression and the clinic pathological fe atures of SLE were further analyzed. DNA methylation was analyzed by bisulfite sequencing PCR (BSP). Our data indicated that the level of CXCL14 in the PBMC of SLE patients was statistically lower than that in healthy controls (P < 0.05). Further analysis showed that CXCL14 expression was negatively correlated with anti-Sj gren syndrome B antibody(anti-SSB antibody, P < 0.01) and albuminuria(P < 0.05). However, CXCL14 expression was not significantly correlated with the indexes of SLE activity, renal damage, the level of anti-ds-DNA antibodies, complement C3 and C-reactive protein. In addition, we further demonstrated that the CXCL14 promoter hypermethylation expres-sion was significant higher than healthy controls. Down-regulated of CXCL14 expression in PBMC maybe involved in the occur-rence or development of SLE disease. The loss of CXCL14 expression was regulated by promoter hypermethylation.