BACKGROUND Systemic lupus erythematosus(SLE)is the most frequent and serious systemic connective tissue disease.Nowadays there is no clear guidance on its treatment in childhood.There are a lot of negative effects of ...BACKGROUND Systemic lupus erythematosus(SLE)is the most frequent and serious systemic connective tissue disease.Nowadays there is no clear guidance on its treatment in childhood.There are a lot of negative effects of standard-of-care treatment(SOCT),including steroid toxicity.Rituximab(RTX)is the biological B-lymphocyte-depleting agent suggested as a basic therapy in pediatric SLE.AIM To compare the benefits of RTX above SOCT.METHODS The data from case histories of 79 children from the Saint-Petersburg State Pediatric Medical University from 2012 to 2022 years,were analyzed.The diagnosis of SLE was established with SLICC criteria.We compared the outcomes of treatment of SLE in children treated with and without RTX.Laboratory data,doses of glucocorticosteroids,disease activity measured with SELENA-SLEDAI,RESULTS Patients,treated with RTX initially had a higher degree of disease activity with prevalence of central nervous system and kidney involvement,compared to patients with SOCT.One year later the disease characteristics became similar between groups with a more marked reduction of disease activity(SELENA-SLEDAI activity index)in the children who received RTX[-19 points(17;23)since baseline]compared to children with SOCT[-10(5;15.5)points since baseline,P=0.001],the number of patients with active lupus nephritis,and daily proteinuria.During RTX therapy,infectious diseases had three patients;one patient developed a bi-cytopenia.CONCLUSION RTX can be considered as the option in the treatment of severe forms of SLE,due to its ability to arrest disease activity compared to SOCT.展开更多
Macrophage activation syndrome (MAS), in its secondary form, often complicates rheumatic diseases but rarely constitutes a mode of revelation. Systemic lupus erythematosus (SLE) is a systemic autoimmune disease of unk...Macrophage activation syndrome (MAS), in its secondary form, often complicates rheumatic diseases but rarely constitutes a mode of revelation. Systemic lupus erythematosus (SLE) is a systemic autoimmune disease of unknown etiology that primarily affects women in adulthood. MAS is a serious condition that may be the first presentation of SLE. Here, we report the case of a 4-year-old female with MAS as the primary manifestation of Systemic Lupus Erythematosus (SLE). In this case, we outline the characteristics of a complex case of SLE that was initially accompanied with MAS, and also review the literature to discuss the clinical, biological, and therapeutic aspects of this condition.展开更多
BACKGROUND Hemophagocytic lymphohistiocytosis(HLH) is a rare life-threatening disorder,often resulting in the immune-mediated injury of multiple organ systems,including primary HLH and secondary HLH(sHLH). Among them,...BACKGROUND Hemophagocytic lymphohistiocytosis(HLH) is a rare life-threatening disorder,often resulting in the immune-mediated injury of multiple organ systems,including primary HLH and secondary HLH(sHLH). Among them, sHLH results from infections, malignant, or autoimmune conditions, which have quite poor outcomes even with aggressive management and are more common in adults.CASE SUMMARY We report a rare case of a 36-year-old female manifested with sHLH on background with systemic lupus erythematosus(SLE). During hospitalization, the patient was characterized by recurrent high-grade fever, petechiae and ecchymoses of abdominal skin, and pulmonary infection. Whole exon gene sequencing revealed decreased activity of natural killer cells. She received systematic treatment with Methylprednisolone, Etoposide, and anti-infective drugs. Intravenous immunoglobulin and plasmapheresis were applied when the condition was extremely acute and progressive. The patient recovered and did not present any relapse of the HLH for one year of follow-up.CONCLUSION The case showed sHLH, thrombotic microvascular, and infection in the whole course of the disease, which was rarely reported by now. The treatment of the patient emphasizes that early recognition and treatment of sHLH in SLE patients was of utmost importance to improve the prognosis and survival rate of patients.展开更多
Lupus nephritis(LN) has a high incidence in systemic lupus erythematosus(SLE) patients, but there is a lack of sensitive predictive markers. The purpose of the study was to investigate the association between the CD4^...Lupus nephritis(LN) has a high incidence in systemic lupus erythematosus(SLE) patients, but there is a lack of sensitive predictive markers. The purpose of the study was to investigate the association between the CD4^(+)CD8^(+)double positive T(DPT) lymphocytes and LN. The study included patients with SLE without renal impairment(SLE-NRI), LN, nephritic syndrome(NS), or nephritis. Peripheral blood lymphocyte subsets were analyzed by flow cytometry. Biochemical measurements were performed with peripheral blood in accordance with the recommendations proposed by the National Center for Clinical Laboratories. The proportions of DPT cells in the LN group were significantly higher than that in the SLE-NRI group(t=4.012, P<0.001), NS group(t=3.240,P=0.001), and nephritis group(t=2.57, P=0.011). In the LN group, the risk of renal impairment increased significantly in a DPT cells proportion-dependent manner. The risk of LN was 5.136 times(95% confidence interval, 2.115–12.473) higher in cases with a high proportion of DPT cells than those whose proportion of DPT cells within the normal range. These findings indicated that the proportion of DPT cells could be a potential marker to evaluate LN susceptibility, and the interference of NS and nephritis could be effectively excluded when assessing the risk of renal impairment during SLE with DPT cell proportion.展开更多
BACKGROUND Multicentric reticulohistiocytosis(MRH)/systemic lupus erythematosus(SLE) overlap syndrome is an uncommon disease in the clinic and is diagnosed through characteristic clinical manifestations, histopatholog...BACKGROUND Multicentric reticulohistiocytosis(MRH)/systemic lupus erythematosus(SLE) overlap syndrome is an uncommon disease in the clinic and is diagnosed through characteristic clinical manifestations, histopathology, and immunopathology. Here, we report the case of a 30-year-old woman with SLE who developed MRH.CASE SUMMARY A 30-year-old woman with a history of polyarthritis for the past 12 years had multiple skin nodules on her body for 10 years, including the sacrococcygeal area, dorsum of the hands, interphalangeal joint of the feet and sternoclavicular joint. The histopathology of a biopsy of the distal interphalangeal joint of the hands revealed granulomatous inflammation, fibrous hyperplasia with ground-glass degeneration, inflammatory cell exudation and focal necrosis. The immunohistochemical stains showed positive staining for CD68 and negative staining for S100 and acid-fast staining. The patient was diagnosed with SLE with MRH. Her symptoms were improved after a combined treatment of prednisone, hydroxychloroquine and cyclophosphamide.CONCLUSION MRH/SLE overlap syndrome is difficult to diagnose and treat. Cyclophosphamide may be an alternative choice for the treatment of MRH.展开更多
BACKGROUND Lupus mesenteric vasculitis(LMV)is a serious condition that may occur as an acute manifestation of gastrointestinal(GI)involvement and is not easily diagnosed by physicians.Delayed diagnosis and treatment o...BACKGROUND Lupus mesenteric vasculitis(LMV)is a serious condition that may occur as an acute manifestation of gastrointestinal(GI)involvement and is not easily diagnosed by physicians.Delayed diagnosis and treatment of LMV may lead to rapid disease progression and can be life threatening.CASE SUMMARY A previously healthy 27-year-old woman presented with abdominal pain following a history of fatigue and consumption of cold water.Laboratory invest-igations,physical examinations,and enhanced abdominal computed tomography(CT)suggested systemic lupus erythematosus complicated by LMV.She received treatments,such as GI decompression,somatostatin,glucocorticoids,and immu-nosuppressants,and was evaluated using color ultrasonography.Twenty days later,the patient reported no stomach discomfort and was able to consume semi-liquid food.Laboratory investigations showed that inflammatory factors decreased to normal levels and complement levels increased slightly.One year after discharged,she recovered with methylprednisolone being tapered to 4 mg per day,mycophenolate mofetil to 0.75 g bid,and hydroxychloroquine to 0.2 g bid;however,only C3 complement level was slightly below the normal level.CONCLUSION Early diagnosis of LMV is essential for successful treatment;this depends on a combination of clinical manifestations,laboratory investigations,and imaging findings.Enhanced CT is preferred,but ultrasonography can be used for prompt screening and follow-up.展开更多
BACKGROUND Pediatric-onset systemic lupus erythematosus(SLE)is typically more severe than adult-onset SLE,with a higher incidence of nervous system involvement.Chorea is a relatively rare neurological complication rep...BACKGROUND Pediatric-onset systemic lupus erythematosus(SLE)is typically more severe than adult-onset SLE,with a higher incidence of nervous system involvement.Chorea is a relatively rare neurological complication reported in 2.4%-7%of SLE patients.In particular,chorea induced by glucocorticoid dose reduction is even rarer.Herein,we report the case of a girl with SLE,who developed chorea during the process of glucocorticoid therapy reduction.CASE SUMMARY We describe a 14-year-old girl who was diagnosed with SLE.She was treated with methylprednisolone and rituximab,and her symptoms improved.On the second day after the methylprednisolone dose was reduced according to the treatment guidelines,the patient developed chorea.Her condition improved after adjusting her glucocorticoid regimen.CONCLUSION This case is a reminder that extra attention to chorea is required in SLE patients during glucocorticoid dose reduction.展开更多
Introduction: Little is known about the feasibility and effectiveness of rehabilitative treatment for systemic lupus erythematosus (SLE) in individuals with progressive multifocal leukoencephalopathy (PML). We describ...Introduction: Little is known about the feasibility and effectiveness of rehabilitative treatment for systemic lupus erythematosus (SLE) in individuals with progressive multifocal leukoencephalopathy (PML). We describe a patient with SLE complicated by PML and ameliorated by comprehensive rehabilitation. We also review the epidemiology, pathology, imaging characteristics, and treatment of PML. Patient Concerns: We found a patient with SLE with PML improved by multidisciplinary rehabilitation techniques. Diagnoses, Interventions, and Outcomes: We diagnosed a PML with a 13-year history of SLE and lupus nephritis after longtime immunosuppressive therapy. The patient underwent a comprehensive, multifaceted rehabilitation program, including drug therapy, integrated physical therapy, occupational therapy, acupuncture, music therapy, computer-aided cognitive rehabilitation training, and behavioral management training. This rehabilitation program improved her motor function and activities of daily living. Conclusions: Her condition improved in the short term through comprehensive rehabilitation, including physical, speech, and cognitive therapy. Therefore, we recommend comprehensive rehabilitation to improve the function and activities of daily living in patients with PML.展开更多
BACKGROUND Systemic lupus erythematosus(SLE)is a chronic autoimmune disorder impacting multiple organs,influenced by genetic factors,especially those related to the immune system.However,there is a need for new biomar...BACKGROUND Systemic lupus erythematosus(SLE)is a chronic autoimmune disorder impacting multiple organs,influenced by genetic factors,especially those related to the immune system.However,there is a need for new biomarkers in SLE.MicroRNA-125a(miR-125a)levels are decreased in T cells,B cells,and dendritic cells of SLE patients.MiR-125a plays a regulatory role in controlling the levels of tumor necrosis factor-alpha(TNF-α)and interleukin 12(IL-12),which are crucial pro-inflammatory cytokines in SLE pathogenesis.AIM To assess the levels of miR-125a,IL-12,and TNF-αin SLE patients’plasma,evaluating their diagnostic and prognostic value.METHODS The study included 100 healthy individuals,50 newly diagnosed(ND),and 50 SLE patients undergoing treatment.The patients were monitored for a duration of 24 wk to observe and record instances of relapses.MiR-125a expression was measured using real-time reverse transcription polymerase chain reaction,while ELISA kits were used to assess IL-12 and TNF-αproduction.RESULTS The results showed significantly reduced miR-125a expression in SLE patients compared to healthy individuals,with the lowest levels in ND patients.TNF-αand IL-12 expression levels were significantly elevated in SLE patients,especially in the early stages of the disease.Receiver operating characteristic curve analyses,and Cox-Mantel Log-rank tests indicated miR-125a,TNF-α,and IL-12 as proper diagnostic biomarkers for SLE.A negative correlation was found between plasma miR-125a expression and IL-12/TNF-αlevels in SLE patients.CONCLUSION Decreased miR-125a levels may be involved in the development of SLE,while elevated levels of IL-12 and TNF-αcontribute to immune dysregulation.These findings offer new diagnostic and prognostic markers for SLE.Moreover,the negative correlation observed suggests an interaction between miR-125a,TNF-α,and IL-12.Further research is necessary to uncover the underlying mechanisms that govern these relationships.展开更多
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
Background:Systemic lupus erythematosus(SLE)is a complex chronic autoimmune disease with no known cure.However,the regulatory mechanism of immunity-related genes is not fully understood in SLE.In order to explore new ...Background:Systemic lupus erythematosus(SLE)is a complex chronic autoimmune disease with no known cure.However,the regulatory mechanism of immunity-related genes is not fully understood in SLE.In order to explore new therapeutic targets,we used bioinformatical methods to analyze a series of data.Methods:After downloading and processing the data from Gene Expression Omnibus database,the differentially expressed genes of SLE were analyzed.CIBERSORT algorithm was used to analyze the immune infiltration of SLE.Based on single-cell RNA-sequencing data,the role of immune-related genes in SLE and its target organ(kidney)were analyzed.Key transcription factors affecting immune-related genes were identified.Cell-cell communication networks in SLE were analyzed.Results:In total,15 hub genes and 4 transcription factors were found in the bulk data.Monocytes and macrophages in GSE81622(SLE)showed more infiltration.There were four cell types were annotated in scRNA sequencing dataset(GSE135779),as follows T cells,monocyte,NK cells and B cells.Immunity-related genes were overexpressed in monocytes.Conclusion:The present study shows that immune-related genes affect SLE through monocytes and play an important role in target organ renal injury.展开更多
BACKGROUND Pregnancy in the setting of systemic lupus erythematosus can worsen thecondition from the stable to active stage, with quality of life and fertility desirebeing particular concerns. Pregnancy in the active ...BACKGROUND Pregnancy in the setting of systemic lupus erythematosus can worsen thecondition from the stable to active stage, with quality of life and fertility desirebeing particular concerns. Pregnancy in the active stage of systemic lupuserythematosus (ASLE), although rare and complicated to manage, can be treatedfavorably with immunotherapies ifs used properly. Here we report such a successcase.CASE SUMMARY A 31-year-old primigravida patient, diagnosed with SLE seven years ago, wasinduced ASLE after a cold at 21 + weeks. The patient’s vital signs on presentationwere normal. Her laboratory exam was remarkable for significant proteinuria,liver and renal dysfunction, and low C3 and C4 levels. Infectious work-up wasnegative. The patient was diagnosed with ASLE. She was given immunosuppressiveagents (methylprednisolone, gamma globulin and azathioprine etc.)and plasma adsorption therapy, monitoring blood pressure every 8 h, fetal heartrate twice a day, and liver and renal function at least twice a week. Successfulmaternal and fetal outcomes are presented here.CONCLUSION Child-bearing in ASLE has become more promising, even for this difficult case ofASLE with multiple organ damage. Thorough antepartum counseling, cautiousmaternal-fetal monitoring, and multi-organ function monitoring bymultidisciplinary specialties are keys to favorable pregnancy outcomes.展开更多
Systemic lupus erythematosus (SLE) is an autoimmune inflammatory disease characterized by the presence of a plethora of autoantibodies and immune complex formation. Virtually every system and organ can be affected by ...Systemic lupus erythematosus (SLE) is an autoimmune inflammatory disease characterized by the presence of a plethora of autoantibodies and immune complex formation. Virtually every system and organ can be affected by SLE. Gastrointestinal symptoms are com-mon in SLE patients, and more than half of them are caused by adverse reactions to medications and viral or bacterial infections. Though not as common as lu-pus nephritis, SLE-related gastrointestinal involvement is clinically important because most cases can be life-threatening if not treated promptly. Lupus mesenteric vasculitis is the most common cause, followed by pro-tein-losing enteropathy, intestinal pseudo-obstruction, acute pancreatitis and other rare complications such as celiac disease, inflammatory bowel diseases, etc. No specific autoantibody is identified as being associated with SLE-related gastroenteropathy. Imaging studies, particularly abdominal computed tomography scans, are helpful in diagnosing some SLE-related gastroen-teropathies. Most of these complications have good therapeutic responses to corticosteroids and immu-nosuppressive agents. Supportive measures such as bowel rest, nutritional support, antibiotics and proki-netic medications are helpful in facilitating functional recovery and improving the outcome.展开更多
The immunophenotyping expression levels of lymphocyte in the peripheral blood from 21 patients with active systemic lupus erythematosus were analyzed by using the immunofluorescence labeling flow cytometry technique t...The immunophenotyping expression levels of lymphocyte in the peripheral blood from 21 patients with active systemic lupus erythematosus were analyzed by using the immunofluorescence labeling flow cytometry technique to investigate the immunophenotyping expression of lymphocytes T and B in the peripheral blood of active SLE patients and its clinical value. It was showed that, compared with normal controls, the expression of CD + 3, CD + 4 and the ratio of CD + 4/CD + 8 in the peripheral blood of these patients were decreased , while the expression of CD + 8, CD + 20 was significantly increased . It was suggested that both T and B cells in patients with active SLE involved in immunoregulation, were activated. The abnormal expression of lymphocyte immunophenotyping could influence the immune reaction in SLE patients, which might be one of the important pathogenesis factors in SLE.展开更多
Objective:This study examined patients with systemic lupus erythematosus(SLE)as a means to explore the impacts of chronic disease on life courses.Methods:A semi-structured in-depth interviews were conducted with nine ...Objective:This study examined patients with systemic lupus erythematosus(SLE)as a means to explore the impacts of chronic disease on life courses.Methods:A semi-structured in-depth interviews were conducted with nine patients in China in 2017;participants were included based on their having or having had facial butterfly erythema.Results:The study focused on both the disease's impact on the patients'health and on their daily lives.Four core themes emerged:visible changes in the patient's bodies,social dilemmas,"the encouragement of disease",and a new perspective on the relationship between disease and health.One important finding was that the occurrence of a chronic disease did not have only negative repercussions;some patients felt that there were advantages to being sick.Chronic disease resulted in a reworking of daily life.The patients developed a self-referential model of healing.Conclusion:The distinct interpretations of the same disease offered by different patients served to yield a more complete understanding of the disease.People with SLE adjust their thinking about the disease based on personal feelings as well as experiences and pursue a dialogue on their illness based on the disease pattern unique to them.The meaning that disease had for the patients was not limited to negative connotations.展开更多
BACKGROUND Systemic lupus erythematosus(SLE)and antineutrophil cytoplasmic antibodyassociated vasculitis(AAV)are classically thought to cause renal impairment and small vessel vasculitis with different pathophysiologi...BACKGROUND Systemic lupus erythematosus(SLE)and antineutrophil cytoplasmic antibodyassociated vasculitis(AAV)are classically thought to cause renal impairment and small vessel vasculitis with different pathophysiologies.Their overlap constitutes a rare rheumatologic disease.To date,only dozens of such cases with biopsyproven glomerulonephritis have been reported worldwide typically in women of childbearing age.Here,we present a unique clinical case due to its rarity and individualized treatment of a Chinese man in his eighth decade of life.CASE SUMMARY A 77-year-old man was admitted to several hospitals for shortness of breath and received nonspecific treatments over the past 3 years.As his symptoms were not completely relieved,he visited our hospital for further treatment.Laboratory examinations revealed kidney dysfunction,severe anaemia,hypocomplementemia,glomerular proteinuria,and microscopic haematuria.Antinuclear antibodies,as well as anti-dsDNA antibodies,were positive.Computed tomography of the chest showed right pleural effusion.Renal biopsy was performed,and histology suggested crescentic glomerulonephritis,pauci-immune type.After treatment with plasmapheresis,glucocorticoid,and cyclophosphamide,the disease was in remission,and the patient remained in a stable condition for over 3 years post-hospital discharge.CONCLUSION Due to its complexity and rarity,SLE and AAV overlap syndrome is easily misdiagnosed.An accurate diagnosis and treatment at the earliest stage may significantly improve the condition and reduce irreversible organ injury.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 To investigate the effects of salvianolic acid A(SAA)in systemic lupus erythematosus(SLE)induced by pristane in BALB/c mice,this study was performed.METHODS Lupus mice were established by confirming elevated...OBJECTIVE To investigate the effects of salvianolic acid A(SAA)in systemic lupus erythematosus(SLE)induced by pristane in BALB/c mice,this study was performed.METHODS Lupus mice were established by confirming elevated levels of autoantibodies and IL-6 after intraperitoneal injection of pristane.Micewere then treated with daily oral doses of SAA for 5months in parallel with mice treated with prednisone and aspirin as positive controls.The levels of autoantibodies were monitored at monthly intervals and nephritic symptoms observed by hematoxylin and eosin(H&E)and periodic acid-Schiff(PAS)staining.Western blot analysis of renal tissue was also employed.RESULTS SAA treatment caused a significant reduction in the levels of anti-Sm autoantibodies and reduced renal histopathological changes and pathological effects.SAA treatment also significantly inhibited the phosphorylation of IKK,IκB and NFκB in renal tissues of lupus mice.CONCLUSION The results suggest that SAA alleviates renal injury in pristane-induced SLE in BALB/c mice through inhibition of phosphorylation of IKK,IκB and NFκB.展开更多
Objective MicroRNAs are fine regulators for gene expression during the post-transcriptional stage in many autoimmune diseases.HypoxamiRs(miR-210 and miR-21)play an important role in hypoxia and in inflammation-associa...Objective MicroRNAs are fine regulators for gene expression during the post-transcriptional stage in many autoimmune diseases.HypoxamiRs(miR-210 and miR-21)play an important role in hypoxia and in inflammation-associated hypoxia.Systemic lupus erythematosus(SLE)is a chronic systemic autoimmune disease that would potentiate many pathological complications,including hemolytic anemia.This study aimed to investigate the role of hypoxamiRs in SLE/hemolytic anemia patients.Methods This work was designed to analyze the circulating levels of↱the miR-210 and miR-21 expressions and hypoxia-inducible factor-1α(HIF-α)in SLE/hemolytic anemia patients.SLE activity was evaluated for all patients by SLE Disease Activity Index(SLEDAI).Clinical manifestations/complications and serological/hematological investigations were reported.HIF-αconcentration was assayed by ELISA and expression of miR-21 and miR-210 was analyzed by qRT-PCR.Results The results indicated that the fold change of the miR-210/miR-21 expressions in plasma was significantly elevated in SLE/hemolytic anemia patients.A strong positive correlation between the miR-210 and miR-21 expression levels was also recorded.Among the associated-disease complications,hypertension,arthritis,oral ulcers,and serositis were associated with a high circulating miR-210 expression,while the occurrence of renal disorders was associated with the increased miR-21 expression.Furthermore,the HIF-αlevel was remarkably elevated in SLE/hemolytic anemia patients.A high positive correlation was recorded between the HIF-αconcentration and miR-210/miR-21 expression levels.The occurrence of oral ulcers,arthritis,and hypertension was associated with the increased HIF-αconcentration.On the other hand,SLEDAI and white blood cell count were positively correlated with miR-21/miR-210.The erythrocyte sedimentation rate was positively correlated with miR-21.Conclusion The dysregulation of the circulating miR-210/miR-210/HIF-1αlevels in SLE/hemolytic anemia patients advocated that the hypoxia pathway might have an essential role in the pathogenesis and complications of these diseases.展开更多
In a recent article on World J Clin Cases 2019;7:3859-3865,Sun et al reported a case of 36-year-old female with macrophage activity syndrome as an onset of systemic lupus erythematosus.Although this is a very interest...In a recent article on World J Clin Cases 2019;7:3859-3865,Sun et al reported a case of 36-year-old female with macrophage activity syndrome as an onset of systemic lupus erythematosus.Although this is a very interesting case,some concerns still need to be addressed.First,the patient had an extremely elevated serum ferritin but a normal C-reactive protein level,which was unparallel with the inflammatory condition before she received any treatments.Second,the diagnosis of systemic lupus erythematosus seemed to be insufficient according to the patient’s medical information presented,most of which were not specific to lupus but could be explained by macrophage activity syndrome.Hence,more medical information on the patient should be provided,and a profound discussion needs to be addressed.展开更多
基金Supported by the Ministry of Science and Higher Education of the Russian Federation,No.075-15-2022-301the Russian Science Foundation,No.22-45-08004.
文摘BACKGROUND Systemic lupus erythematosus(SLE)is the most frequent and serious systemic connective tissue disease.Nowadays there is no clear guidance on its treatment in childhood.There are a lot of negative effects of standard-of-care treatment(SOCT),including steroid toxicity.Rituximab(RTX)is the biological B-lymphocyte-depleting agent suggested as a basic therapy in pediatric SLE.AIM To compare the benefits of RTX above SOCT.METHODS The data from case histories of 79 children from the Saint-Petersburg State Pediatric Medical University from 2012 to 2022 years,were analyzed.The diagnosis of SLE was established with SLICC criteria.We compared the outcomes of treatment of SLE in children treated with and without RTX.Laboratory data,doses of glucocorticosteroids,disease activity measured with SELENA-SLEDAI,RESULTS Patients,treated with RTX initially had a higher degree of disease activity with prevalence of central nervous system and kidney involvement,compared to patients with SOCT.One year later the disease characteristics became similar between groups with a more marked reduction of disease activity(SELENA-SLEDAI activity index)in the children who received RTX[-19 points(17;23)since baseline]compared to children with SOCT[-10(5;15.5)points since baseline,P=0.001],the number of patients with active lupus nephritis,and daily proteinuria.During RTX therapy,infectious diseases had three patients;one patient developed a bi-cytopenia.CONCLUSION RTX can be considered as the option in the treatment of severe forms of SLE,due to its ability to arrest disease activity compared to SOCT.
文摘Macrophage activation syndrome (MAS), in its secondary form, often complicates rheumatic diseases but rarely constitutes a mode of revelation. Systemic lupus erythematosus (SLE) is a systemic autoimmune disease of unknown etiology that primarily affects women in adulthood. MAS is a serious condition that may be the first presentation of SLE. Here, we report the case of a 4-year-old female with MAS as the primary manifestation of Systemic Lupus Erythematosus (SLE). In this case, we outline the characteristics of a complex case of SLE that was initially accompanied with MAS, and also review the literature to discuss the clinical, biological, and therapeutic aspects of this condition.
文摘BACKGROUND Hemophagocytic lymphohistiocytosis(HLH) is a rare life-threatening disorder,often resulting in the immune-mediated injury of multiple organ systems,including primary HLH and secondary HLH(sHLH). Among them, sHLH results from infections, malignant, or autoimmune conditions, which have quite poor outcomes even with aggressive management and are more common in adults.CASE SUMMARY We report a rare case of a 36-year-old female manifested with sHLH on background with systemic lupus erythematosus(SLE). During hospitalization, the patient was characterized by recurrent high-grade fever, petechiae and ecchymoses of abdominal skin, and pulmonary infection. Whole exon gene sequencing revealed decreased activity of natural killer cells. She received systematic treatment with Methylprednisolone, Etoposide, and anti-infective drugs. Intravenous immunoglobulin and plasmapheresis were applied when the condition was extremely acute and progressive. The patient recovered and did not present any relapse of the HLH for one year of follow-up.CONCLUSION The case showed sHLH, thrombotic microvascular, and infection in the whole course of the disease, which was rarely reported by now. The treatment of the patient emphasizes that early recognition and treatment of sHLH in SLE patients was of utmost importance to improve the prognosis and survival rate of patients.
基金supported by the Natural Science Foundation of Sichuan Province (Grant No.2022NSFSC1415)the Special Project of Sichuan Province Traditional Chinese Medicine Administration (Grant No. 2020JC0124)+1 种基金the Management Project of General Hospital of Western Theater Command (Grants No. 2021-XZYG-C22 and 2021-XZYG-C21)the Spark Young Innovative Talent Project of General Hospital of Western Theater Command。
文摘Lupus nephritis(LN) has a high incidence in systemic lupus erythematosus(SLE) patients, but there is a lack of sensitive predictive markers. The purpose of the study was to investigate the association between the CD4^(+)CD8^(+)double positive T(DPT) lymphocytes and LN. The study included patients with SLE without renal impairment(SLE-NRI), LN, nephritic syndrome(NS), or nephritis. Peripheral blood lymphocyte subsets were analyzed by flow cytometry. Biochemical measurements were performed with peripheral blood in accordance with the recommendations proposed by the National Center for Clinical Laboratories. The proportions of DPT cells in the LN group were significantly higher than that in the SLE-NRI group(t=4.012, P<0.001), NS group(t=3.240,P=0.001), and nephritis group(t=2.57, P=0.011). In the LN group, the risk of renal impairment increased significantly in a DPT cells proportion-dependent manner. The risk of LN was 5.136 times(95% confidence interval, 2.115–12.473) higher in cases with a high proportion of DPT cells than those whose proportion of DPT cells within the normal range. These findings indicated that the proportion of DPT cells could be a potential marker to evaluate LN susceptibility, and the interference of NS and nephritis could be effectively excluded when assessing the risk of renal impairment during SLE with DPT cell proportion.
文摘BACKGROUND Multicentric reticulohistiocytosis(MRH)/systemic lupus erythematosus(SLE) overlap syndrome is an uncommon disease in the clinic and is diagnosed through characteristic clinical manifestations, histopathology, and immunopathology. Here, we report the case of a 30-year-old woman with SLE who developed MRH.CASE SUMMARY A 30-year-old woman with a history of polyarthritis for the past 12 years had multiple skin nodules on her body for 10 years, including the sacrococcygeal area, dorsum of the hands, interphalangeal joint of the feet and sternoclavicular joint. The histopathology of a biopsy of the distal interphalangeal joint of the hands revealed granulomatous inflammation, fibrous hyperplasia with ground-glass degeneration, inflammatory cell exudation and focal necrosis. The immunohistochemical stains showed positive staining for CD68 and negative staining for S100 and acid-fast staining. The patient was diagnosed with SLE with MRH. Her symptoms were improved after a combined treatment of prednisone, hydroxychloroquine and cyclophosphamide.CONCLUSION MRH/SLE overlap syndrome is difficult to diagnose and treat. Cyclophosphamide may be an alternative choice for the treatment of MRH.
文摘BACKGROUND Lupus mesenteric vasculitis(LMV)is a serious condition that may occur as an acute manifestation of gastrointestinal(GI)involvement and is not easily diagnosed by physicians.Delayed diagnosis and treatment of LMV may lead to rapid disease progression and can be life threatening.CASE SUMMARY A previously healthy 27-year-old woman presented with abdominal pain following a history of fatigue and consumption of cold water.Laboratory invest-igations,physical examinations,and enhanced abdominal computed tomography(CT)suggested systemic lupus erythematosus complicated by LMV.She received treatments,such as GI decompression,somatostatin,glucocorticoids,and immu-nosuppressants,and was evaluated using color ultrasonography.Twenty days later,the patient reported no stomach discomfort and was able to consume semi-liquid food.Laboratory investigations showed that inflammatory factors decreased to normal levels and complement levels increased slightly.One year after discharged,she recovered with methylprednisolone being tapered to 4 mg per day,mycophenolate mofetil to 0.75 g bid,and hydroxychloroquine to 0.2 g bid;however,only C3 complement level was slightly below the normal level.CONCLUSION Early diagnosis of LMV is essential for successful treatment;this depends on a combination of clinical manifestations,laboratory investigations,and imaging findings.Enhanced CT is preferred,but ultrasonography can be used for prompt screening and follow-up.
基金This study was conducted with the approval of the ethics committee of Chongqing Hospital of Traditional Chinese Medicine(approval No.2023-GAKY-KS-XYQ).The patient gave explicit informed consent to report her clinical case.
文摘BACKGROUND Pediatric-onset systemic lupus erythematosus(SLE)is typically more severe than adult-onset SLE,with a higher incidence of nervous system involvement.Chorea is a relatively rare neurological complication reported in 2.4%-7%of SLE patients.In particular,chorea induced by glucocorticoid dose reduction is even rarer.Herein,we report the case of a girl with SLE,who developed chorea during the process of glucocorticoid therapy reduction.CASE SUMMARY We describe a 14-year-old girl who was diagnosed with SLE.She was treated with methylprednisolone and rituximab,and her symptoms improved.On the second day after the methylprednisolone dose was reduced according to the treatment guidelines,the patient developed chorea.Her condition improved after adjusting her glucocorticoid regimen.CONCLUSION This case is a reminder that extra attention to chorea is required in SLE patients during glucocorticoid dose reduction.
文摘Introduction: Little is known about the feasibility and effectiveness of rehabilitative treatment for systemic lupus erythematosus (SLE) in individuals with progressive multifocal leukoencephalopathy (PML). We describe a patient with SLE complicated by PML and ameliorated by comprehensive rehabilitation. We also review the epidemiology, pathology, imaging characteristics, and treatment of PML. Patient Concerns: We found a patient with SLE with PML improved by multidisciplinary rehabilitation techniques. Diagnoses, Interventions, and Outcomes: We diagnosed a PML with a 13-year history of SLE and lupus nephritis after longtime immunosuppressive therapy. The patient underwent a comprehensive, multifaceted rehabilitation program, including drug therapy, integrated physical therapy, occupational therapy, acupuncture, music therapy, computer-aided cognitive rehabilitation training, and behavioral management training. This rehabilitation program improved her motor function and activities of daily living. Conclusions: Her condition improved in the short term through comprehensive rehabilitation, including physical, speech, and cognitive therapy. Therefore, we recommend comprehensive rehabilitation to improve the function and activities of daily living in patients with PML.
基金the Department of Research and Technology at Golestan University of Medical Sciences,No.113017.
文摘BACKGROUND Systemic lupus erythematosus(SLE)is a chronic autoimmune disorder impacting multiple organs,influenced by genetic factors,especially those related to the immune system.However,there is a need for new biomarkers in SLE.MicroRNA-125a(miR-125a)levels are decreased in T cells,B cells,and dendritic cells of SLE patients.MiR-125a plays a regulatory role in controlling the levels of tumor necrosis factor-alpha(TNF-α)and interleukin 12(IL-12),which are crucial pro-inflammatory cytokines in SLE pathogenesis.AIM To assess the levels of miR-125a,IL-12,and TNF-αin SLE patients’plasma,evaluating their diagnostic and prognostic value.METHODS The study included 100 healthy individuals,50 newly diagnosed(ND),and 50 SLE patients undergoing treatment.The patients were monitored for a duration of 24 wk to observe and record instances of relapses.MiR-125a expression was measured using real-time reverse transcription polymerase chain reaction,while ELISA kits were used to assess IL-12 and TNF-αproduction.RESULTS The results showed significantly reduced miR-125a expression in SLE patients compared to healthy individuals,with the lowest levels in ND patients.TNF-αand IL-12 expression levels were significantly elevated in SLE patients,especially in the early stages of the disease.Receiver operating characteristic curve analyses,and Cox-Mantel Log-rank tests indicated miR-125a,TNF-α,and IL-12 as proper diagnostic biomarkers for SLE.A negative correlation was found between plasma miR-125a expression and IL-12/TNF-αlevels in SLE patients.CONCLUSION Decreased miR-125a levels may be involved in the development of SLE,while elevated levels of IL-12 and TNF-αcontribute to immune dysregulation.These findings offer new diagnostic and prognostic markers for SLE.Moreover,the negative correlation observed suggests an interaction between miR-125a,TNF-α,and IL-12.Further research is necessary to uncover the underlying mechanisms that govern these relationships.
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Background:Systemic lupus erythematosus(SLE)is a complex chronic autoimmune disease with no known cure.However,the regulatory mechanism of immunity-related genes is not fully understood in SLE.In order to explore new therapeutic targets,we used bioinformatical methods to analyze a series of data.Methods:After downloading and processing the data from Gene Expression Omnibus database,the differentially expressed genes of SLE were analyzed.CIBERSORT algorithm was used to analyze the immune infiltration of SLE.Based on single-cell RNA-sequencing data,the role of immune-related genes in SLE and its target organ(kidney)were analyzed.Key transcription factors affecting immune-related genes were identified.Cell-cell communication networks in SLE were analyzed.Results:In total,15 hub genes and 4 transcription factors were found in the bulk data.Monocytes and macrophages in GSE81622(SLE)showed more infiltration.There were four cell types were annotated in scRNA sequencing dataset(GSE135779),as follows T cells,monocyte,NK cells and B cells.Immunity-related genes were overexpressed in monocytes.Conclusion:The present study shows that immune-related genes affect SLE through monocytes and play an important role in target organ renal injury.
文摘BACKGROUND Pregnancy in the setting of systemic lupus erythematosus can worsen thecondition from the stable to active stage, with quality of life and fertility desirebeing particular concerns. Pregnancy in the active stage of systemic lupuserythematosus (ASLE), although rare and complicated to manage, can be treatedfavorably with immunotherapies ifs used properly. Here we report such a successcase.CASE SUMMARY A 31-year-old primigravida patient, diagnosed with SLE seven years ago, wasinduced ASLE after a cold at 21 + weeks. The patient’s vital signs on presentationwere normal. Her laboratory exam was remarkable for significant proteinuria,liver and renal dysfunction, and low C3 and C4 levels. Infectious work-up wasnegative. The patient was diagnosed with ASLE. She was given immunosuppressiveagents (methylprednisolone, gamma globulin and azathioprine etc.)and plasma adsorption therapy, monitoring blood pressure every 8 h, fetal heartrate twice a day, and liver and renal function at least twice a week. Successfulmaternal and fetal outcomes are presented here.CONCLUSION Child-bearing in ASLE has become more promising, even for this difficult case ofASLE with multiple organ damage. Thorough antepartum counseling, cautiousmaternal-fetal monitoring, and multi-organ function monitoring bymultidisciplinary specialties are keys to favorable pregnancy outcomes.
文摘Systemic lupus erythematosus (SLE) is an autoimmune inflammatory disease characterized by the presence of a plethora of autoantibodies and immune complex formation. Virtually every system and organ can be affected by SLE. Gastrointestinal symptoms are com-mon in SLE patients, and more than half of them are caused by adverse reactions to medications and viral or bacterial infections. Though not as common as lu-pus nephritis, SLE-related gastrointestinal involvement is clinically important because most cases can be life-threatening if not treated promptly. Lupus mesenteric vasculitis is the most common cause, followed by pro-tein-losing enteropathy, intestinal pseudo-obstruction, acute pancreatitis and other rare complications such as celiac disease, inflammatory bowel diseases, etc. No specific autoantibody is identified as being associated with SLE-related gastroenteropathy. Imaging studies, particularly abdominal computed tomography scans, are helpful in diagnosing some SLE-related gastroen-teropathies. Most of these complications have good therapeutic responses to corticosteroids and immu-nosuppressive agents. Supportive measures such as bowel rest, nutritional support, antibiotics and proki-netic medications are helpful in facilitating functional recovery and improving the outcome.
文摘The immunophenotyping expression levels of lymphocyte in the peripheral blood from 21 patients with active systemic lupus erythematosus were analyzed by using the immunofluorescence labeling flow cytometry technique to investigate the immunophenotyping expression of lymphocytes T and B in the peripheral blood of active SLE patients and its clinical value. It was showed that, compared with normal controls, the expression of CD + 3, CD + 4 and the ratio of CD + 4/CD + 8 in the peripheral blood of these patients were decreased , while the expression of CD + 8, CD + 20 was significantly increased . It was suggested that both T and B cells in patients with active SLE involved in immunoregulation, were activated. The abnormal expression of lymphocyte immunophenotyping could influence the immune reaction in SLE patients, which might be one of the important pathogenesis factors in SLE.
文摘Objective:This study examined patients with systemic lupus erythematosus(SLE)as a means to explore the impacts of chronic disease on life courses.Methods:A semi-structured in-depth interviews were conducted with nine patients in China in 2017;participants were included based on their having or having had facial butterfly erythema.Results:The study focused on both the disease's impact on the patients'health and on their daily lives.Four core themes emerged:visible changes in the patient's bodies,social dilemmas,"the encouragement of disease",and a new perspective on the relationship between disease and health.One important finding was that the occurrence of a chronic disease did not have only negative repercussions;some patients felt that there were advantages to being sick.Chronic disease resulted in a reworking of daily life.The patients developed a self-referential model of healing.Conclusion:The distinct interpretations of the same disease offered by different patients served to yield a more complete understanding of the disease.People with SLE adjust their thinking about the disease based on personal feelings as well as experiences and pursue a dialogue on their illness based on the disease pattern unique to them.The meaning that disease had for the patients was not limited to negative connotations.
文摘BACKGROUND Systemic lupus erythematosus(SLE)and antineutrophil cytoplasmic antibodyassociated vasculitis(AAV)are classically thought to cause renal impairment and small vessel vasculitis with different pathophysiologies.Their overlap constitutes a rare rheumatologic disease.To date,only dozens of such cases with biopsyproven glomerulonephritis have been reported worldwide typically in women of childbearing age.Here,we present a unique clinical case due to its rarity and individualized treatment of a Chinese man in his eighth decade of life.CASE SUMMARY A 77-year-old man was admitted to several hospitals for shortness of breath and received nonspecific treatments over the past 3 years.As his symptoms were not completely relieved,he visited our hospital for further treatment.Laboratory examinations revealed kidney dysfunction,severe anaemia,hypocomplementemia,glomerular proteinuria,and microscopic haematuria.Antinuclear antibodies,as well as anti-dsDNA antibodies,were positive.Computed tomography of the chest showed right pleural effusion.Renal biopsy was performed,and histology suggested crescentic glomerulonephritis,pauci-immune type.After treatment with plasmapheresis,glucocorticoid,and cyclophosphamide,the disease was in remission,and the patient remained in a stable condition for over 3 years post-hospital discharge.CONCLUSION Due to its complexity and rarity,SLE and AAV overlap syndrome is easily misdiagnosed.An accurate diagnosis and treatment at the earliest stage may significantly improve the condition and reduce irreversible organ injury.
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.
基金The project supported by National Natural Science Foundation of China(81573645,81673422)
文摘OBJECTIVE To investigate the effects of salvianolic acid A(SAA)in systemic lupus erythematosus(SLE)induced by pristane in BALB/c mice,this study was performed.METHODS Lupus mice were established by confirming elevated levels of autoantibodies and IL-6 after intraperitoneal injection of pristane.Micewere then treated with daily oral doses of SAA for 5months in parallel with mice treated with prednisone and aspirin as positive controls.The levels of autoantibodies were monitored at monthly intervals and nephritic symptoms observed by hematoxylin and eosin(H&E)and periodic acid-Schiff(PAS)staining.Western blot analysis of renal tissue was also employed.RESULTS SAA treatment caused a significant reduction in the levels of anti-Sm autoantibodies and reduced renal histopathological changes and pathological effects.SAA treatment also significantly inhibited the phosphorylation of IKK,IκB and NFκB in renal tissues of lupus mice.CONCLUSION The results suggest that SAA alleviates renal injury in pristane-induced SLE in BALB/c mice through inhibition of phosphorylation of IKK,IκB and NFκB.
基金supported by the Taif University Researchers Supporting Project(No.TURSP-2020/103).
文摘Objective MicroRNAs are fine regulators for gene expression during the post-transcriptional stage in many autoimmune diseases.HypoxamiRs(miR-210 and miR-21)play an important role in hypoxia and in inflammation-associated hypoxia.Systemic lupus erythematosus(SLE)is a chronic systemic autoimmune disease that would potentiate many pathological complications,including hemolytic anemia.This study aimed to investigate the role of hypoxamiRs in SLE/hemolytic anemia patients.Methods This work was designed to analyze the circulating levels of↱the miR-210 and miR-21 expressions and hypoxia-inducible factor-1α(HIF-α)in SLE/hemolytic anemia patients.SLE activity was evaluated for all patients by SLE Disease Activity Index(SLEDAI).Clinical manifestations/complications and serological/hematological investigations were reported.HIF-αconcentration was assayed by ELISA and expression of miR-21 and miR-210 was analyzed by qRT-PCR.Results The results indicated that the fold change of the miR-210/miR-21 expressions in plasma was significantly elevated in SLE/hemolytic anemia patients.A strong positive correlation between the miR-210 and miR-21 expression levels was also recorded.Among the associated-disease complications,hypertension,arthritis,oral ulcers,and serositis were associated with a high circulating miR-210 expression,while the occurrence of renal disorders was associated with the increased miR-21 expression.Furthermore,the HIF-αlevel was remarkably elevated in SLE/hemolytic anemia patients.A high positive correlation was recorded between the HIF-αconcentration and miR-210/miR-21 expression levels.The occurrence of oral ulcers,arthritis,and hypertension was associated with the increased HIF-αconcentration.On the other hand,SLEDAI and white blood cell count were positively correlated with miR-21/miR-210.The erythrocyte sedimentation rate was positively correlated with miR-21.Conclusion The dysregulation of the circulating miR-210/miR-210/HIF-1αlevels in SLE/hemolytic anemia patients advocated that the hypoxia pathway might have an essential role in the pathogenesis and complications of these diseases.
文摘In a recent article on World J Clin Cases 2019;7:3859-3865,Sun et al reported a case of 36-year-old female with macrophage activity syndrome as an onset of systemic lupus erythematosus.Although this is a very interesting case,some concerns still need to be addressed.First,the patient had an extremely elevated serum ferritin but a normal C-reactive protein level,which was unparallel with the inflammatory condition before she received any treatments.Second,the diagnosis of systemic lupus erythematosus seemed to be insufficient according to the patient’s medical information presented,most of which were not specific to lupus but could be explained by macrophage activity syndrome.Hence,more medical information on the patient should be provided,and a profound discussion needs to be addressed.
