Invasive fungal infections have grown significantly over the last two decades, owing to an increase in immunocompromised hosts and geriatric patients. When the host’s defenses are compromised, such infections are ass...Invasive fungal infections have grown significantly over the last two decades, owing to an increase in immunocompromised hosts and geriatric patients. When the host’s defenses are compromised, such infections are associated with severe morbidity and mortality. Here, a rare case of fungal infection in a 61-year-old immunocompetent male patient from Saudi Arabia was reported, who suffered from pulmonary hemorrhage and Systemic Lupus Erythematous. Bronchoalveolar Lavage was used as a diagnostic tool to identify the fungus reported in the case. The pathogenic fungal specie identified as Magnusiomyces capitatus, in macroscopic and microscopic morphological characteristics of the colonies. Based on clinical evidence, liposomal amphotericin formulation was recommended for initial therapy against fungal infection. Also, liposomal amphotericin B induced mycological eradication up to 70 percent in patients with proven Magnusiomyces capitatus infection. In addition to addressing suspected Systemic lupus erythematosus, the patient’s health has improved with no evidence of pulmonary bleeding and hemoptysis.展开更多
In this paper</span><span style="font-family:Verdana;">,</span><span style="font-family:Verdana;"> we present a thorough review of one of the most</span><span style...In this paper</span><span style="font-family:Verdana;">,</span><span style="font-family:Verdana;"> we present a thorough review of one of the most</span><span style="font-family:Verdana;"> life-threatening autoimmune diseases, Systemic lupus erythematosus (lupus). Symptoms, risk factors, including genetic and epidemiological factors are discussed. Treatment, life expectancies, and Health Related Quality of Life of patients with SLE will be discussed as well. Special attention will be given to Lupus Nephritis.展开更多
Objective:Observe the clinical characteristics of children with SLE,namely,to observe the symptoms and laboratory examinations,such as blood routine,blood lipid,immunoglobulin,complement,autoantibodies,serum 25(OH)D a...Objective:Observe the clinical characteristics of children with SLE,namely,to observe the symptoms and laboratory examinations,such as blood routine,blood lipid,immunoglobulin,complement,autoantibodies,serum 25(OH)D and other indicators,and to explore the clinical characteristics,the difference and the significance of vitamin D supplements between male and female SLE patients in children respectively.Methods:We enrolled 64 cases of SLE patients in children who were admitted into the department of pediatrics and rheumatology of the third affiliated hospital of sun yat-sen university in guangzhou from May 1,2011 to February 1,2019,They were analyzed retrospectively,adoptingΧ²test for statistical analysis.Results:64 cases of SLE in children,which included 10 cases of male and 54 cases of female.Clinical manifestations:facial skin rash in 48 patients(75%),fever in 38 cases(59.4%),arthritis in 28 cases(43.8%),oral ulcer in 18 cases(28.1%),serositis in13 cases(20.3%),and the sun allergy in 9 cases(14.1%),the damage of central nervous system in 7 cases(10.9%).Laboratory examination:30 cases of leukopenia(46.9%),anemia in 30 cases(46.9%),thrombocytopenia in 12 cases(18.8%),hematuria in 18 cases(28.1%),proteinuria in 33 cases(51.2%),6 patients with renal impairment(9.4%),antinuclear antibody positive in 63 cases(98.4%),anti-double-stranded DNA(dsDNA)antibody positive in 48 cases(75%),anti SSA antibody positive in 44 cases(68.7%),SSB antibody positive in 33 cases(51.6%),Sm antibody positive in 40 cases(62.5%),nucleosome antibody positive in 28 cases(43.8%).Among these children,male SLE patients were higher than female children with SLE in the damage of kidney,Sm antibodies and resisting nucleosome antibody positive rates(Χ²=4.451,8.336,6.803,P<0.05),the female children with SLE was higher than male SLE Children in the anti-SSB antibody positive rate(Χ²=4.945,P<0.05).In 64 cases of SLE children,which included 52 cases were lower than the normal level of serum 25(OH)D measurements,12 cases were in the normal lower limit of serum 25(OH)D measurements,at the same time,the female SLE.Patients was higher than male children with SLE in the reduce rate of serum 25(OH)D(Χ²=8.351,P<0.05).Conclusion:Male SLE patients which appeared damage of kidney easier than female patients,the proteinuria was the most common in the damage of kidney.Resistance to Sm antibodies which was the risk factor of renal injury with higher incidence in male children with SLE;Anti nucleosome antibody which was the risk factor for the disease activity in male children with SLE were higher than female children with SLE.It was estimated that the risk of Sjogren’s syndrome appeared in female with SLE were higher than that in male SLE children.In this retrospective study,the serum 25(OH)D levels were significantly lower in children with SLE,and vitamin D supplementation was required.展开更多
The role played by cytokines, other than interferon (IFN)-a, in the differentiation and function of dendritic cells (DCs) in systemic lupus erythematosus (SLE), remains unclear. Serum interleukin-10 (IL-10) le...The role played by cytokines, other than interferon (IFN)-a, in the differentiation and function of dendritic cells (DCs) in systemic lupus erythematosus (SLE), remains unclear. Serum interleukin-10 (IL-10) levels are generally elevated in SLE patients, which might modulate the differentiation of DCs. In this study, DCs were induced from monocytes either by transendothelial trafficking or by culture with granulocyte-macrophage colony-stimulating factor (GM-CSF) + IL-4 + tumor necrosis factor (TNF)-a. Both systems were used to investigate the effects of elevated serum IL-10 level on DC differentiation in SLE patients. The results showed that monocyte-derived DCs induced by either SLE serum or exogenous IL-10 reduced the expression of human leukocyte antigen (HLA)-DR and CD80, decreased IL-12p40 level, and increased IL-10 level, and exhibited an impaired capacity to stimulate allogenic T-cell proliferation. These results indicate that serum IL-10 may be involved in the pathogenesis of SLE by modulating the differentiation and function of DCs.展开更多
BACKGROUND: There are fewer reports on systemic lupus erythematosus (SLE) related myelitis, and definite and uniform therapeutic program is not available. OBJECTIVE: To observe the clinical manifestations, imaging...BACKGROUND: There are fewer reports on systemic lupus erythematosus (SLE) related myelitis, and definite and uniform therapeutic program is not available. OBJECTIVE: To observe the clinical manifestations, imaging characteristics, results of laboratory examination and treatment of SLE. DESIGN: A retrospective case analysis. SETTING: Department of Neurology, the Second Affiliated Hospital of Sun Yat-sen University. PARTICIPANTS: Totally 1 052 SLE inpatients were selected from the Second Affiliated Hospital of Sun Yat-sen University from January 1995 to May 2005, and they all accorded with the diagnostic standards for SLE set by American Rheumatism Association in 1982. 124 of them were diagnosed to have damage of central nervous system. Inclusive criteria: Patients with one of the focal physical signs, including mental and behavior disorders, headache, seizure and involvement of nervous system. Exclusive criteria: Patients with hypertensive encephalopathy, damage of nervous system due to uremia and infection of central nervous system. Spinal cord lesion occurred in 15 female cases of 23 - 51 years old. Informed consents were obtained from all the participants. METHODS: The physical signs, laboratory examinations, therapeutic program and prognosis were recorded in the 15 patients with symptoms of spinal cord lesions. All the patients underwent MRI scan of brain or lesioned segment of spinal cord, and 8 cases of them underwent lumbar puncture to determine intracranial pressure, routine and biochemical examinations were cerebrospinal fluid were performed. The disease activity of SLE in systems beyond central nervous system was evaluated with modified lupus activity criteria count (LACC). MAIN OUTCOME MEASURES:① Incidence of SLE related myelitis, attack age distribution and its association with the activity of SLE; ② Comparisons of the clinical characteristics, cranial and spinal cord MRI manifestations, different therapeutic program and prognosis. RESULTS: All the 15 SLE patients were involved in the analysis of results. ① The incidence of SLE related myelitis was low (1%, 15/1 052). ②SLE related myelitis occurred mostly when the SLE symptoms were active, and only a few occurred at the stable period. ③ Among the SLE patients, MRI displayed abnormal changes in 71% (10/14), the typical changes appeared abnormal signals at corresponding spinal segments, manifested as prolonged T1 and T2 signals, thickened spinal segments. Lumbar segments were mostly involved. ④ Of the 9 patients treated with hormone impact, 7 cases (78%) had obvious improvements, and the effects were better in those treated with immunosuppressor combined with intravenous immunoglobulin of large dosage. CONCLUSION:① Myelitis is a rare complication of SLE.② MRI serves as a valuable supplementary approach in the diagnosis of SLE related myelitis without specificity. ③ Steroid pulse combined with immunosuppressor and intravenous immunoglobulin of large dosage is effective in the treatment.展开更多
We present a retrospective review of DNA immunoadsorption (DNA-IA) therapy on clinical symptoms as well as indicators in pediatric cases with systemic lupus erythematosus (SLE), and follow up the short-term curative e...We present a retrospective review of DNA immunoadsorption (DNA-IA) therapy on clinical symptoms as well as indicators in pediatric cases with systemic lupus erythematosus (SLE), and follow up the short-term curative effects. 16 SLE cases were treated by DNA-IA for 3 times every other day. We observed the changes on clinical manifestations and immunological indicators, in order to compare the alteration of these indicators including clinical manifestations, Systemic Lupus Erythematosus Disease Active Index (SLEDAI) scores, 24 hurinary protein excretion, autoantibodies, serum IgG and complement C3. 13 cases were followed up regularly, within 3 months after DNA-IA therapy, 12 cases of clinical manifestations improved (92.3%). SLEDAI scores in 10 cases decreased from (16.20 ± 12.54) to less than 5 (76.9%), 8 cases of ANA, anti-DNA antibodies were negative (61.5%), 13 cases with IgG level in serum recovered to normal (10.39 ± 4.38) g/L, C3 level rose to normal (1.06 ± 0.23) g/L. 3 to 6 months after IA, clinical manifestations and laboratory examinations in all cases got maximum improved. 9 months after IA, SLEDAI score in 2 cases (15.4%) rose to more than 5, anti-DNA antibody in 2 cases (15.4%) became positive, and 1case (7.7%) with serum C3 decreased again. 2 cases died from multiple organs dysfunction within 3 to 6 months after IA. No serious complications were found during DNA-IA. We recommend that DNA immunoadsorption is a safe and effective therapy for active childhood-onset SLE, which could improve clinical symptoms, eliminate ANA and anti-DNA antibodies. Combining with corticosteroids and immunosuppressive drugs, DNA-IA could significantly reduce the activity of disease and protect vital organs function in the short term.展开更多
Systemic lupus erythematosus(SLE) is a typical autoimmune disease. Lymphotoxin β receptor(LTβR) signaling plays an important role in autoimmune inflammations. LTβR-Ig fusion protein, LTβR blocking agent, has b...Systemic lupus erythematosus(SLE) is a typical autoimmune disease. Lymphotoxin β receptor(LTβR) signaling plays an important role in autoimmune inflammations. LTβR-Ig fusion protein, LTβR blocking agent, has been used to treat SLE, while its mechanism remains to be fully elucidated. In this study, to investigate the expression of LTβR in the T cells of SLE patients and its roles in the pathogenesis of SLE, we isolated the peripheral blood T cells of SLE patients and normal controls to detect expression of LTβR by flow cytometry and RNA assay. T cells were also stimulated with LIGHT, a ligand of LTβR, and then detected for their LTβR expressions and apoptosis by flow cytometry. Also, their expressions of inflammatory factors and receptors were determined by RNA assay. The results showed that LTβR positive cells were 22.75%±6.98% in CD3~+ cells of SLE patients, while there were almost no LTβR positive cells in CD3~+ cells of normal persons. Moreover, LTβR expression was remarkably higher in CD3,CD4 and CD8 positive T cells of active SLE patients than non/low active patients(all P〈0.05), and positively correlated with increased Ig level, decreased complement level and renal damage. Moreover, the stimulation of SLE T cells with LIGHT promoted higher expression of LTβR, IL-23 R and IL-17 A, and apoptosis of T cells. In conclusion,we demonstrated a high expression of LTβR in the T cells of SLE patients which may be associated with pathogenesis of SLE.展开更多
Anti-phospholipid antibodies (APA) like anti-cardiolipin antibodies (ACA) and anti-β2glycoprotien (anti-β2GP) are important cause of venous and arterial thrombosis and other occlusive vascular diseases. The prevalen...Anti-phospholipid antibodies (APA) like anti-cardiolipin antibodies (ACA) and anti-β2glycoprotien (anti-β2GP) are important cause of venous and arterial thrombosis and other occlusive vascular diseases. The prevalence of these antibodies in SLE patients at the time of diagnosis is not known in Indian SLE patients. This study was conducted to evaluate the prevalence of ACA and anti-β2GP autoantibodies in SLE patients and to correlate them with disease activity and immune parameters such as C3, C4 and CRP levels. where 85 SLE patients referred from Rheumatology Department, KEM hospital, Mumbai were studied. SLE disease activity was evaluated by SLE Disease Activity Index (SLEDAI) score at the time of evaluation. All patients studied were in an active stage of disease of which 37.6% patients had renal disorders, which were categorized as Lupus Nephritis (LN) and 62.3% patients did not show any renal manifestations (non-LN). ACA and anti-β2GP autoantibodies, to IgG and IgM subclasses were tested by ELISA. C3, C4 and CRP levels were detected by nephelometer. It was observed that 12.9% patients were IgG-ACA and IgM-ACA positive and ACA positivity was noted more among LN group Anti-β2GP autoantibody positivity was 27.1% for IgG and 31.8% for IgM., IgG-anti-β2GP antibodies were slightly higher in non-LN patients, whereas a higher incidence of IgM-anti-β2GP antibodies were detected in LN patients. Hence detection both ACA and anti-β2GP antibodies along with associated immune parameters were helpful to evaluate their possible association with disease severity in SLE patients. A long term follow up of patients having ACA and anti-β2GP antibodies without thrombotic event is also needed to detect their possible thrombotic event in future along with their clinical presentation.展开更多
Purpose: To report a case of atypical Kikuchi-Fujimoto disease (KFD) that illustrates several overlapping features with systemic lupus erythematosus (SLE). Methods: A case is reported followed by a review of the curre...Purpose: To report a case of atypical Kikuchi-Fujimoto disease (KFD) that illustrates several overlapping features with systemic lupus erythematosus (SLE). Methods: A case is reported followed by a review of the current literature. Case report: A 16-year-old boy with an unusual manifestation of Kikuchi-Fujimoto disease (KFD) is described. The patient presented with fever, weight loss and severe abdominal pain, due to extensive necrotizing retroperitoneal and mesenteric lymphadenopathy. During the course of his illness, he developed several symptoms suggestive of systemic lupus erythematosus (SLE): a pericardial effusion, cotton wool spots on the retina and antibodies against nuclear antigens (ANA), Smith (Sm) and ribonucleoprotein (RNP) antigens. However, no additional features of SLE were found. The patient subsequently fully recovered within two months, without initiation of immunosuppressive therapy. His autoantibodies became negative five months after initial presentation and he remains well at his 23 month follow up visit. Discussion: We hypothesize that the autoantibodies developed by our patient were secondary to self-antigen induced autoimmunity related to his extensive tissue necrosis. Despite initially having clinical features suggestive of SLE, our patient’s full and spontaneous recovery strongly supports the diagnosis of KFD. This illustrates the need for careful diagnosis, in order to avoid unnecessary and potentially toxic treatment with immunosuppressive agents.展开更多
Aim: We aimed to investigate whether the agonists for liver X receptor (LXR) ameliorate lupus-like phenotypes in mice mediated by the clearance of apoptotic cells, and compare with peroxisome proliferator-activated re...Aim: We aimed to investigate whether the agonists for liver X receptor (LXR) ameliorate lupus-like phenotypes in mice mediated by the clearance of apoptotic cells, and compare with peroxisome proliferator-activated receptor (PPAR) γ plus PPARδ agonists, which also facilitate the clearance of apoptotic cells and exert anti-inflammatory effects in systemic lupus erythematosus (SLE). Methods: We investigated the efficacy of LXR agonist (GW3965) or dual treatment of PPARγ (pioglitazone) and PPARδ (GW0742) agonists in SLE animal models, female MRL/MpJ-Fas/J mice and BALB/cAJcl mice treated with pristane. The data were analyzed with one-way analysis of variance and Tukey’s honestly significant difference tests. Results: The treatment with LXR or PPARγ/δ agonists did not significantly alter the swelling of lymph nodes, ds-DNA production, albuminuria, histological score of glomerular lesions, and mRNA expression of target genes including Abca1, C1qa, Icam1, Mertk and Tnf. Conclusion: LXR or PPARγ/δ agonists targeting the impaired clearance for apoptosis cells may not be efficient in the remission induction therapy in SLE.展开更多
Background: MicroRNAs (miRs) are noncoding gene regulators that may have a role as diagnostic or prognostic biomarkers in systemic lupus erythematosus (SLE). Aim: To measure the blood levels of miR-146a, miR-126 and m...Background: MicroRNAs (miRs) are noncoding gene regulators that may have a role as diagnostic or prognostic biomarkers in systemic lupus erythematosus (SLE). Aim: To measure the blood levels of miR-146a, miR-126 and miR-30a in Sudanese SLE patients and to investigate their potential role in disease pathogenesis and utility as biomarkers for SLE. Material and Methods: A total of 48 SLE patients and 20 matched healthy individuals were enrolled in this study. SLE disease activity index (SLEDAI) was assessed. The blood levels of miR-146a, miR-126 and miR-30a were determined by Real-time polymerase chain reaction (PCR) in all participants. Γ-INF and IL-2 were analyzed by ELISA, and CD markers were used in flow cytometry. Results: The mean age of the patients was 31.5 ± 8.5 years with disease duration > 5 years. In SLE patients, the mean blood level fold changes of miR-146a (0.33 ± 0.277;P < 0.001), miR-126 (2.44 ± 1.771;P = 0.007) and miR-30a (1.56 ± 1.40;P > 0.305) compared to controls. Down regulation of miR-146a increase expression of γ-INF (P < 0.002), whereas the up regulation of miR-126 increase expression of CD markers (P MiR-126 at a cut-off value 1.209 and miR-146a at cut-off value of 0.9233 which can discriminate between SLE patients significantly associated with SLE disease. Conversely, miR-30a was insignificantly associated with SLE disease (P value > 0.05) as no differences between the SLE patients and healthy control. Conclusion: Circulating miR-146a and miR-126 could be a potential noninvasive biomarker in SLE. This study provides an overview of the current state of research on the role of miRNAs in the immune pathogenesis and regulation of SLE. Further studies are needed in miRNAs profiling expressions of SLE diseases.展开更多
Introduction: Anti-Müllerian hormone (AMH) is shown to be a possible indicator of ovarian function. Severe systemic lupus erythematosus (SLE) patients exposed to high-dose cyclophosphamide (CTX) have a much highe...Introduction: Anti-Müllerian hormone (AMH) is shown to be a possible indicator of ovarian function. Severe systemic lupus erythematosus (SLE) patients exposed to high-dose cyclophosphamide (CTX) have a much higher risk of developing infertility and premature ovarian failure. Therefore, we performed a prospective case-control study to evaluate the impact of SLE on women’s ovarian reserve using AMH before CTX therapy. Methods: SLE patients before receiving CTX therapy were enrolled in our hospital. Age-matched healthy women were served as controls. Serum AMH level was measured using an enzyme-linked immunosorbent assay. Basal hormone levels were measured including follicle-stimulating hormone, luteinizing hormone, and estradiol on the third day of their menstrual periods. All participants underwent transvaginal ultrasonographic examination for the determination of total antral follicle count on the third day. Results: AMH value in SLE patients was significantly lower compared to healthy control with normal ovarian reserve. No significant difference in AMH levels was found between SLE and healthy control with low ovarian reserve. Conclusions: SLE patients not receiving CTX therapy even with normal menstruation, still had an impaired ovarian reserve. Therefore, early monitoring of AMH levels could better reflect the ovarian function and reproductive outcomes of SLE patients and relative protective strategy needed to reserve fertility.展开更多
1 Introduction Neuropsychiatric systemic lupus erythematosus(NPSLE)is a serious complication of systemic lupus erythematosus(SLE),with an incidence of about 30%to 40%[1].No matter early or late SLE patients are prone ...1 Introduction Neuropsychiatric systemic lupus erythematosus(NPSLE)is a serious complication of systemic lupus erythematosus(SLE),with an incidence of about 30%to 40%[1].No matter early or late SLE patients are prone to concurrent,so early diagnosis and treatment of NPSLE is extremely important.展开更多
Macrophages play a pivotal role in systemic lupus erythematosus(SLE)therapy.Efforts have been made to develop multifunctional drug delivery systems capable of directing macrophage polarization.Here,we present a novel ...Macrophages play a pivotal role in systemic lupus erythematosus(SLE)therapy.Efforts have been made to develop multifunctional drug delivery systems capable of directing macrophage polarization.Here,we present a novel hyaluronic acid methacrylate(HAMA)hydrogel microparticle encapsulating multiple cytokines for SLE remission though enhancing macrophage functions.The HAMA microparticles loaded with monocyte chemotactic protein-1(MCP-1)and interleukin-4(IL-4)were fabricated by using a microfluidic technology.The released MCP-1 facilitates the aggregation of inflammatory macrophages,after which IL-4 induces the macrophage phenotype shift from inflammatory M1 to immune-protective M2,thus restoring immune balance.We have demonstrated in MRL/lpr mice that the hydrogel microparticles could improve their efficacy of intraperitoneal drug delivery,modulate immune function,and attenuate the disease symptoms.These results suggest that our proposed microparticles delivery platform has potential clinical value for treating autoimmune diseases.展开更多
<strong>Background: </strong>Lupus nephritis (LN) is one of the most common presentations of Systemic lupus erythematosus (SLE). Cyclophosphamide is one of the key immunosuppressive agents for the manageme...<strong>Background: </strong>Lupus nephritis (LN) is one of the most common presentations of Systemic lupus erythematosus (SLE). Cyclophosphamide is one of the key immunosuppressive agents for the management of LN. Leflunomide is an isoxazole immunomodulatory agent has been shown to be safe, well tolerated and effective in SLE and LN. <strong>Objective: </strong>To evaluate the outcome of leflunomide in the treatment of proliferative lupus nephritis compared to cyclophosphamide. <strong>Method: </strong>This randomized clinical trial was held in Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, Bangladesh from July 2017 to August 2019. A total of 66 patients of proliferative lupus nephritis who need induction therapy were enrolled in this study. Leflunomide 100 mg/day for consecutive 3 days followed by 0.5 mg/kg/day in divided dose was given in experimental group (n = 32) and intravenous cyclophosphamide 0.5 gm/m2 of body surface area monthly pulse was given in control group (n = 34). All study patients have received prednisolone and hydroxychloroquine according to KDIGO guideline then followed up monthly for 6 months. Outcomes were measured at 6th month by renal function [S. Creatinine, 24 hours urinary total protein (24-hr UTP)], changes in SELENA-SLEDAI score, anti-ds DNA level, serum complement levels (serum C3 & C4), remission (complete/partial) and adverse drug responses.<strong> Result:</strong> In experimental group, remission occurred in 18 (56.3%) patients and no remission in 14 (43.7%) patients. In control group, remission occurred in 24 (70.6%) patients and no remission in 10 (29.4%) patients. Adverse effects in experimental group were: elevated ALT (6.3%), hypertension (12.5%), infection (6.3%) and amenorrhea (12.5%). In control group, adverse effects were mainly leucopenia (5.9%), infection (17.7%) and amenorrhea (29.4%). Intergroup analysis for treatment responses and adverse effects showed no significant difference (p > 0.05). <strong>Conclusion:</strong> Leflunomide combined with prednisolone is effective in the induction treatment of proliferative lupus nephritis in Bangladeshi patients in terms of response rate and adverse effects.展开更多
文摘Invasive fungal infections have grown significantly over the last two decades, owing to an increase in immunocompromised hosts and geriatric patients. When the host’s defenses are compromised, such infections are associated with severe morbidity and mortality. Here, a rare case of fungal infection in a 61-year-old immunocompetent male patient from Saudi Arabia was reported, who suffered from pulmonary hemorrhage and Systemic Lupus Erythematous. Bronchoalveolar Lavage was used as a diagnostic tool to identify the fungus reported in the case. The pathogenic fungal specie identified as Magnusiomyces capitatus, in macroscopic and microscopic morphological characteristics of the colonies. Based on clinical evidence, liposomal amphotericin formulation was recommended for initial therapy against fungal infection. Also, liposomal amphotericin B induced mycological eradication up to 70 percent in patients with proven Magnusiomyces capitatus infection. In addition to addressing suspected Systemic lupus erythematosus, the patient’s health has improved with no evidence of pulmonary bleeding and hemoptysis.
文摘In this paper</span><span style="font-family:Verdana;">,</span><span style="font-family:Verdana;"> we present a thorough review of one of the most</span><span style="font-family:Verdana;"> life-threatening autoimmune diseases, Systemic lupus erythematosus (lupus). Symptoms, risk factors, including genetic and epidemiological factors are discussed. Treatment, life expectancies, and Health Related Quality of Life of patients with SLE will be discussed as well. Special attention will be given to Lupus Nephritis.
文摘Objective:Observe the clinical characteristics of children with SLE,namely,to observe the symptoms and laboratory examinations,such as blood routine,blood lipid,immunoglobulin,complement,autoantibodies,serum 25(OH)D and other indicators,and to explore the clinical characteristics,the difference and the significance of vitamin D supplements between male and female SLE patients in children respectively.Methods:We enrolled 64 cases of SLE patients in children who were admitted into the department of pediatrics and rheumatology of the third affiliated hospital of sun yat-sen university in guangzhou from May 1,2011 to February 1,2019,They were analyzed retrospectively,adoptingΧ²test for statistical analysis.Results:64 cases of SLE in children,which included 10 cases of male and 54 cases of female.Clinical manifestations:facial skin rash in 48 patients(75%),fever in 38 cases(59.4%),arthritis in 28 cases(43.8%),oral ulcer in 18 cases(28.1%),serositis in13 cases(20.3%),and the sun allergy in 9 cases(14.1%),the damage of central nervous system in 7 cases(10.9%).Laboratory examination:30 cases of leukopenia(46.9%),anemia in 30 cases(46.9%),thrombocytopenia in 12 cases(18.8%),hematuria in 18 cases(28.1%),proteinuria in 33 cases(51.2%),6 patients with renal impairment(9.4%),antinuclear antibody positive in 63 cases(98.4%),anti-double-stranded DNA(dsDNA)antibody positive in 48 cases(75%),anti SSA antibody positive in 44 cases(68.7%),SSB antibody positive in 33 cases(51.6%),Sm antibody positive in 40 cases(62.5%),nucleosome antibody positive in 28 cases(43.8%).Among these children,male SLE patients were higher than female children with SLE in the damage of kidney,Sm antibodies and resisting nucleosome antibody positive rates(Χ²=4.451,8.336,6.803,P<0.05),the female children with SLE was higher than male SLE Children in the anti-SSB antibody positive rate(Χ²=4.945,P<0.05).In 64 cases of SLE children,which included 52 cases were lower than the normal level of serum 25(OH)D measurements,12 cases were in the normal lower limit of serum 25(OH)D measurements,at the same time,the female SLE.Patients was higher than male children with SLE in the reduce rate of serum 25(OH)D(Χ²=8.351,P<0.05).Conclusion:Male SLE patients which appeared damage of kidney easier than female patients,the proteinuria was the most common in the damage of kidney.Resistance to Sm antibodies which was the risk factor of renal injury with higher incidence in male children with SLE;Anti nucleosome antibody which was the risk factor for the disease activity in male children with SLE were higher than female children with SLE.It was estimated that the risk of Sjogren’s syndrome appeared in female with SLE were higher than that in male SLE children.In this retrospective study,the serum 25(OH)D levels were significantly lower in children with SLE,and vitamin D supplementation was required.
基金supported by grants from Science Research Foundation of Ministry of Education of China (No. 205057)Foundation of Jiangsu Province Natural Science (No. 2004148)
文摘The role played by cytokines, other than interferon (IFN)-a, in the differentiation and function of dendritic cells (DCs) in systemic lupus erythematosus (SLE), remains unclear. Serum interleukin-10 (IL-10) levels are generally elevated in SLE patients, which might modulate the differentiation of DCs. In this study, DCs were induced from monocytes either by transendothelial trafficking or by culture with granulocyte-macrophage colony-stimulating factor (GM-CSF) + IL-4 + tumor necrosis factor (TNF)-a. Both systems were used to investigate the effects of elevated serum IL-10 level on DC differentiation in SLE patients. The results showed that monocyte-derived DCs induced by either SLE serum or exogenous IL-10 reduced the expression of human leukocyte antigen (HLA)-DR and CD80, decreased IL-12p40 level, and increased IL-10 level, and exhibited an impaired capacity to stimulate allogenic T-cell proliferation. These results indicate that serum IL-10 may be involved in the pathogenesis of SLE by modulating the differentiation and function of DCs.
文摘BACKGROUND: There are fewer reports on systemic lupus erythematosus (SLE) related myelitis, and definite and uniform therapeutic program is not available. OBJECTIVE: To observe the clinical manifestations, imaging characteristics, results of laboratory examination and treatment of SLE. DESIGN: A retrospective case analysis. SETTING: Department of Neurology, the Second Affiliated Hospital of Sun Yat-sen University. PARTICIPANTS: Totally 1 052 SLE inpatients were selected from the Second Affiliated Hospital of Sun Yat-sen University from January 1995 to May 2005, and they all accorded with the diagnostic standards for SLE set by American Rheumatism Association in 1982. 124 of them were diagnosed to have damage of central nervous system. Inclusive criteria: Patients with one of the focal physical signs, including mental and behavior disorders, headache, seizure and involvement of nervous system. Exclusive criteria: Patients with hypertensive encephalopathy, damage of nervous system due to uremia and infection of central nervous system. Spinal cord lesion occurred in 15 female cases of 23 - 51 years old. Informed consents were obtained from all the participants. METHODS: The physical signs, laboratory examinations, therapeutic program and prognosis were recorded in the 15 patients with symptoms of spinal cord lesions. All the patients underwent MRI scan of brain or lesioned segment of spinal cord, and 8 cases of them underwent lumbar puncture to determine intracranial pressure, routine and biochemical examinations were cerebrospinal fluid were performed. The disease activity of SLE in systems beyond central nervous system was evaluated with modified lupus activity criteria count (LACC). MAIN OUTCOME MEASURES:① Incidence of SLE related myelitis, attack age distribution and its association with the activity of SLE; ② Comparisons of the clinical characteristics, cranial and spinal cord MRI manifestations, different therapeutic program and prognosis. RESULTS: All the 15 SLE patients were involved in the analysis of results. ① The incidence of SLE related myelitis was low (1%, 15/1 052). ②SLE related myelitis occurred mostly when the SLE symptoms were active, and only a few occurred at the stable period. ③ Among the SLE patients, MRI displayed abnormal changes in 71% (10/14), the typical changes appeared abnormal signals at corresponding spinal segments, manifested as prolonged T1 and T2 signals, thickened spinal segments. Lumbar segments were mostly involved. ④ Of the 9 patients treated with hormone impact, 7 cases (78%) had obvious improvements, and the effects were better in those treated with immunosuppressor combined with intravenous immunoglobulin of large dosage. CONCLUSION:① Myelitis is a rare complication of SLE.② MRI serves as a valuable supplementary approach in the diagnosis of SLE related myelitis without specificity. ③ Steroid pulse combined with immunosuppressor and intravenous immunoglobulin of large dosage is effective in the treatment.
文摘We present a retrospective review of DNA immunoadsorption (DNA-IA) therapy on clinical symptoms as well as indicators in pediatric cases with systemic lupus erythematosus (SLE), and follow up the short-term curative effects. 16 SLE cases were treated by DNA-IA for 3 times every other day. We observed the changes on clinical manifestations and immunological indicators, in order to compare the alteration of these indicators including clinical manifestations, Systemic Lupus Erythematosus Disease Active Index (SLEDAI) scores, 24 hurinary protein excretion, autoantibodies, serum IgG and complement C3. 13 cases were followed up regularly, within 3 months after DNA-IA therapy, 12 cases of clinical manifestations improved (92.3%). SLEDAI scores in 10 cases decreased from (16.20 ± 12.54) to less than 5 (76.9%), 8 cases of ANA, anti-DNA antibodies were negative (61.5%), 13 cases with IgG level in serum recovered to normal (10.39 ± 4.38) g/L, C3 level rose to normal (1.06 ± 0.23) g/L. 3 to 6 months after IA, clinical manifestations and laboratory examinations in all cases got maximum improved. 9 months after IA, SLEDAI score in 2 cases (15.4%) rose to more than 5, anti-DNA antibody in 2 cases (15.4%) became positive, and 1case (7.7%) with serum C3 decreased again. 2 cases died from multiple organs dysfunction within 3 to 6 months after IA. No serious complications were found during DNA-IA. We recommend that DNA immunoadsorption is a safe and effective therapy for active childhood-onset SLE, which could improve clinical symptoms, eliminate ANA and anti-DNA antibodies. Combining with corticosteroids and immunosuppressive drugs, DNA-IA could significantly reduce the activity of disease and protect vital organs function in the short term.
文摘Systemic lupus erythematosus(SLE) is a typical autoimmune disease. Lymphotoxin β receptor(LTβR) signaling plays an important role in autoimmune inflammations. LTβR-Ig fusion protein, LTβR blocking agent, has been used to treat SLE, while its mechanism remains to be fully elucidated. In this study, to investigate the expression of LTβR in the T cells of SLE patients and its roles in the pathogenesis of SLE, we isolated the peripheral blood T cells of SLE patients and normal controls to detect expression of LTβR by flow cytometry and RNA assay. T cells were also stimulated with LIGHT, a ligand of LTβR, and then detected for their LTβR expressions and apoptosis by flow cytometry. Also, their expressions of inflammatory factors and receptors were determined by RNA assay. The results showed that LTβR positive cells were 22.75%±6.98% in CD3~+ cells of SLE patients, while there were almost no LTβR positive cells in CD3~+ cells of normal persons. Moreover, LTβR expression was remarkably higher in CD3,CD4 and CD8 positive T cells of active SLE patients than non/low active patients(all P〈0.05), and positively correlated with increased Ig level, decreased complement level and renal damage. Moreover, the stimulation of SLE T cells with LIGHT promoted higher expression of LTβR, IL-23 R and IL-17 A, and apoptosis of T cells. In conclusion,we demonstrated a high expression of LTβR in the T cells of SLE patients which may be associated with pathogenesis of SLE.
文摘Anti-phospholipid antibodies (APA) like anti-cardiolipin antibodies (ACA) and anti-β2glycoprotien (anti-β2GP) are important cause of venous and arterial thrombosis and other occlusive vascular diseases. The prevalence of these antibodies in SLE patients at the time of diagnosis is not known in Indian SLE patients. This study was conducted to evaluate the prevalence of ACA and anti-β2GP autoantibodies in SLE patients and to correlate them with disease activity and immune parameters such as C3, C4 and CRP levels. where 85 SLE patients referred from Rheumatology Department, KEM hospital, Mumbai were studied. SLE disease activity was evaluated by SLE Disease Activity Index (SLEDAI) score at the time of evaluation. All patients studied were in an active stage of disease of which 37.6% patients had renal disorders, which were categorized as Lupus Nephritis (LN) and 62.3% patients did not show any renal manifestations (non-LN). ACA and anti-β2GP autoantibodies, to IgG and IgM subclasses were tested by ELISA. C3, C4 and CRP levels were detected by nephelometer. It was observed that 12.9% patients were IgG-ACA and IgM-ACA positive and ACA positivity was noted more among LN group Anti-β2GP autoantibody positivity was 27.1% for IgG and 31.8% for IgM., IgG-anti-β2GP antibodies were slightly higher in non-LN patients, whereas a higher incidence of IgM-anti-β2GP antibodies were detected in LN patients. Hence detection both ACA and anti-β2GP antibodies along with associated immune parameters were helpful to evaluate their possible association with disease severity in SLE patients. A long term follow up of patients having ACA and anti-β2GP antibodies without thrombotic event is also needed to detect their possible thrombotic event in future along with their clinical presentation.
文摘Purpose: To report a case of atypical Kikuchi-Fujimoto disease (KFD) that illustrates several overlapping features with systemic lupus erythematosus (SLE). Methods: A case is reported followed by a review of the current literature. Case report: A 16-year-old boy with an unusual manifestation of Kikuchi-Fujimoto disease (KFD) is described. The patient presented with fever, weight loss and severe abdominal pain, due to extensive necrotizing retroperitoneal and mesenteric lymphadenopathy. During the course of his illness, he developed several symptoms suggestive of systemic lupus erythematosus (SLE): a pericardial effusion, cotton wool spots on the retina and antibodies against nuclear antigens (ANA), Smith (Sm) and ribonucleoprotein (RNP) antigens. However, no additional features of SLE were found. The patient subsequently fully recovered within two months, without initiation of immunosuppressive therapy. His autoantibodies became negative five months after initial presentation and he remains well at his 23 month follow up visit. Discussion: We hypothesize that the autoantibodies developed by our patient were secondary to self-antigen induced autoimmunity related to his extensive tissue necrosis. Despite initially having clinical features suggestive of SLE, our patient’s full and spontaneous recovery strongly supports the diagnosis of KFD. This illustrates the need for careful diagnosis, in order to avoid unnecessary and potentially toxic treatment with immunosuppressive agents.
文摘Aim: We aimed to investigate whether the agonists for liver X receptor (LXR) ameliorate lupus-like phenotypes in mice mediated by the clearance of apoptotic cells, and compare with peroxisome proliferator-activated receptor (PPAR) γ plus PPARδ agonists, which also facilitate the clearance of apoptotic cells and exert anti-inflammatory effects in systemic lupus erythematosus (SLE). Methods: We investigated the efficacy of LXR agonist (GW3965) or dual treatment of PPARγ (pioglitazone) and PPARδ (GW0742) agonists in SLE animal models, female MRL/MpJ-Fas/J mice and BALB/cAJcl mice treated with pristane. The data were analyzed with one-way analysis of variance and Tukey’s honestly significant difference tests. Results: The treatment with LXR or PPARγ/δ agonists did not significantly alter the swelling of lymph nodes, ds-DNA production, albuminuria, histological score of glomerular lesions, and mRNA expression of target genes including Abca1, C1qa, Icam1, Mertk and Tnf. Conclusion: LXR or PPARγ/δ agonists targeting the impaired clearance for apoptosis cells may not be efficient in the remission induction therapy in SLE.
文摘Background: MicroRNAs (miRs) are noncoding gene regulators that may have a role as diagnostic or prognostic biomarkers in systemic lupus erythematosus (SLE). Aim: To measure the blood levels of miR-146a, miR-126 and miR-30a in Sudanese SLE patients and to investigate their potential role in disease pathogenesis and utility as biomarkers for SLE. Material and Methods: A total of 48 SLE patients and 20 matched healthy individuals were enrolled in this study. SLE disease activity index (SLEDAI) was assessed. The blood levels of miR-146a, miR-126 and miR-30a were determined by Real-time polymerase chain reaction (PCR) in all participants. Γ-INF and IL-2 were analyzed by ELISA, and CD markers were used in flow cytometry. Results: The mean age of the patients was 31.5 ± 8.5 years with disease duration > 5 years. In SLE patients, the mean blood level fold changes of miR-146a (0.33 ± 0.277;P < 0.001), miR-126 (2.44 ± 1.771;P = 0.007) and miR-30a (1.56 ± 1.40;P > 0.305) compared to controls. Down regulation of miR-146a increase expression of γ-INF (P < 0.002), whereas the up regulation of miR-126 increase expression of CD markers (P MiR-126 at a cut-off value 1.209 and miR-146a at cut-off value of 0.9233 which can discriminate between SLE patients significantly associated with SLE disease. Conversely, miR-30a was insignificantly associated with SLE disease (P value > 0.05) as no differences between the SLE patients and healthy control. Conclusion: Circulating miR-146a and miR-126 could be a potential noninvasive biomarker in SLE. This study provides an overview of the current state of research on the role of miRNAs in the immune pathogenesis and regulation of SLE. Further studies are needed in miRNAs profiling expressions of SLE diseases.
文摘Introduction: Anti-Müllerian hormone (AMH) is shown to be a possible indicator of ovarian function. Severe systemic lupus erythematosus (SLE) patients exposed to high-dose cyclophosphamide (CTX) have a much higher risk of developing infertility and premature ovarian failure. Therefore, we performed a prospective case-control study to evaluate the impact of SLE on women’s ovarian reserve using AMH before CTX therapy. Methods: SLE patients before receiving CTX therapy were enrolled in our hospital. Age-matched healthy women were served as controls. Serum AMH level was measured using an enzyme-linked immunosorbent assay. Basal hormone levels were measured including follicle-stimulating hormone, luteinizing hormone, and estradiol on the third day of their menstrual periods. All participants underwent transvaginal ultrasonographic examination for the determination of total antral follicle count on the third day. Results: AMH value in SLE patients was significantly lower compared to healthy control with normal ovarian reserve. No significant difference in AMH levels was found between SLE and healthy control with low ovarian reserve. Conclusions: SLE patients not receiving CTX therapy even with normal menstruation, still had an impaired ovarian reserve. Therefore, early monitoring of AMH levels could better reflect the ovarian function and reproductive outcomes of SLE patients and relative protective strategy needed to reserve fertility.
基金Guangdong Obers Blood Purification Academician Work station(2013B090400004)Construction of collaborative platform for clinical research and clinical research of blood purifica tion(201604020175)+2 种基金Guangzhou entrepreneurial leader talent/LCY201215Guangdong University blood purification technology and Engineering Re search Center(GCZX-A1104)Guangdong Provincial Center for clinical engineering of blood purification(507204531040)
文摘1 Introduction Neuropsychiatric systemic lupus erythematosus(NPSLE)is a serious complication of systemic lupus erythematosus(SLE),with an incidence of about 30%to 40%[1].No matter early or late SLE patients are prone to concurrent,so early diagnosis and treatment of NPSLE is extremely important.
基金supported by the National Key Research and Development Program of China(No.2020YFA0908200)the Key Program of National Natural Science Foundation of China(Nos.81930043,and 82330055)the National Natural Science Foundation of China(Nos.52073060,and 81720108022).
文摘Macrophages play a pivotal role in systemic lupus erythematosus(SLE)therapy.Efforts have been made to develop multifunctional drug delivery systems capable of directing macrophage polarization.Here,we present a novel hyaluronic acid methacrylate(HAMA)hydrogel microparticle encapsulating multiple cytokines for SLE remission though enhancing macrophage functions.The HAMA microparticles loaded with monocyte chemotactic protein-1(MCP-1)and interleukin-4(IL-4)were fabricated by using a microfluidic technology.The released MCP-1 facilitates the aggregation of inflammatory macrophages,after which IL-4 induces the macrophage phenotype shift from inflammatory M1 to immune-protective M2,thus restoring immune balance.We have demonstrated in MRL/lpr mice that the hydrogel microparticles could improve their efficacy of intraperitoneal drug delivery,modulate immune function,and attenuate the disease symptoms.These results suggest that our proposed microparticles delivery platform has potential clinical value for treating autoimmune diseases.
文摘<strong>Background: </strong>Lupus nephritis (LN) is one of the most common presentations of Systemic lupus erythematosus (SLE). Cyclophosphamide is one of the key immunosuppressive agents for the management of LN. Leflunomide is an isoxazole immunomodulatory agent has been shown to be safe, well tolerated and effective in SLE and LN. <strong>Objective: </strong>To evaluate the outcome of leflunomide in the treatment of proliferative lupus nephritis compared to cyclophosphamide. <strong>Method: </strong>This randomized clinical trial was held in Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, Bangladesh from July 2017 to August 2019. A total of 66 patients of proliferative lupus nephritis who need induction therapy were enrolled in this study. Leflunomide 100 mg/day for consecutive 3 days followed by 0.5 mg/kg/day in divided dose was given in experimental group (n = 32) and intravenous cyclophosphamide 0.5 gm/m2 of body surface area monthly pulse was given in control group (n = 34). All study patients have received prednisolone and hydroxychloroquine according to KDIGO guideline then followed up monthly for 6 months. Outcomes were measured at 6th month by renal function [S. Creatinine, 24 hours urinary total protein (24-hr UTP)], changes in SELENA-SLEDAI score, anti-ds DNA level, serum complement levels (serum C3 & C4), remission (complete/partial) and adverse drug responses.<strong> Result:</strong> In experimental group, remission occurred in 18 (56.3%) patients and no remission in 14 (43.7%) patients. In control group, remission occurred in 24 (70.6%) patients and no remission in 10 (29.4%) patients. Adverse effects in experimental group were: elevated ALT (6.3%), hypertension (12.5%), infection (6.3%) and amenorrhea (12.5%). In control group, adverse effects were mainly leucopenia (5.9%), infection (17.7%) and amenorrhea (29.4%). Intergroup analysis for treatment responses and adverse effects showed no significant difference (p > 0.05). <strong>Conclusion:</strong> Leflunomide combined with prednisolone is effective in the induction treatment of proliferative lupus nephritis in Bangladeshi patients in terms of response rate and adverse effects.