Dry eye disease in systemic lupus erythematosus: a cross sectional study

AIM:To explore ocular surface manifestations of dry eye disease(DED)and its influencing factors in systemic lupus erythematosus(SLE)patients.METHODS:Ophthalmological examinations were conducted in SLE patients(n=43)and controls(n=41),including Ocular Surface Disease Index(OSDI),objective scatter index(OSI),tear meniscus height(TMH),lipid layer thickness(LLT),non-invasive Keratograph tear breakup time(NIKBUT),corneal fluorescein score(CFS),Schirmer I test.DED was diagnosed according to the Tear Film and Ocular Surface Society Dry Eye Workshop II Criteria.SLE patients were further divided into DED group and non-DED group,the disease activity,clinical manifestations and laboratory investigations were compared between the two groups.The disease activity was evaluated by Systemic Lupus Erythematosus Disease Activity Index 2000(SLEDAI-2K).Receiver operative characteristic(ROC)curve and multiplefactor binary logistic regression were performed.RESULTS:SLE patients showed higher OSDI[9.1(2.8-15.9)vs 6.3(2.2-7.5),P=0.035],higher OSI[1.67(1.09-2.60)vs 0.96(0.87-1.60),P=0.001],higher CFS[1(0-2)vs 0(0-1),P=0.001],lower LLT[65(42-100)vs 100(79.5-100),P=0.010],and lower NIKBUT[8.03(4.02-9.73)vs 9.67(5.26-12.71),P=0.030]than controls.The 32.6%of SLE patients had DED,which was higher than 12.2%of healthy controls.DED group showed higher SLEDAI-2K score[9.7±6.1 vs 5.4±3.4,P=0.025],higher anti-cardiolipin antibody(ACL)[8.7(3.5-13.2)vs 3.6(2.0-6.9),P=0.035],and higher proportion of patients with cutaneous eruption[42.9%vs 6.9%,P=0.015]than non-DED group.According to multiple-factor binary logistic regression analysis,the SLEDAI-2K score(OR=1.194,P=0.041)and cutaneous eruption(OR=7.094,P=0.045)could be consider as risk factors for DED in SLE patients.The ROC curve of the combined factors including age,disease duration,SLEDAI-2K score,ACL,and cutaneous eruption was analyzed,with a sensitivity of 0.786,a specificity of 0.793,and an area under curve of 0.820.CONCLUSION:Ocular surface affection is frequent in SLE patients,and patients with high disease activity and cutaneous eruption show increased risk of DED.

Refractory autoimmune hemolytic anemia in a patient with systemic lupus erythematosus and ulcerative colitis:A case report

BACKGROUND Ulcerative colitis(UC)and systemic lupus erythematosus(SLE)are both systemic immunoreactive diseases,and their pathogenesis depends on the interaction between genes and environmental factors.There are no reports of UC with SLE in China,but six cases of SLE with UC have been reported in China.The combination of these two diseases has distinct effects on the pathogenesis of both diseases.CASE SUMMARY A female patient(30 years old)came to our hospital due to dull umbilical pain,diarrhea and mucous bloody stool in August 2018 and was diagnosed with UC.The symptoms were relieved after oral administration of mesalazine(1 g po tid)or folic acid(5 mg po qd),and the patient were fed a control diet.On June 24,2019,the patient was admitted for treatment due to anemia and tinnitus.During hospitalization,the patient had repeated low-grade fever and a progressively decreased Hb level.Blood tests revealed positive antinuclear antibody test,positive anti-dsDNA antibody,0.24 g/L C3(0.9-1.8 g/L),0.04 g/L C4(0.1-0.4 g/L),32.37 g/L immunoglobulin(8-17 g/L),and 31568.1 mg/24 h total 24-h urine protein(0-150 mg/24 h).The patient was diagnosed with SLE involving the joints,kidneys and blood system.Previously reported cases of SLE were retrieved from PubMed to characterize clinicopathological features and identify prognostic factors for SLE.CONCLUSION The patient was discharged in remission after a series of treatments,such as intravenous methylprednisolone sodium succinate,intravenous human immunoglobulin,cyclophosphamide injection,and plasma exchange.After discharge,the patient took oral prednisone acetate tablets,cyclosporine capsules,hydroxychloroquine sulfate tablets and other treatments for symptoms and was followed up regularly for 1 month,after which the patient's condition continued to improve and stabilize.

Analysis of status and influencing factors of mental health in patients with systemic lupus erythematosus

BACKGROUND Systemic lupus erythematosus(SLE)is a heterogeneous autoimmune disorder with varied clinical courses and prognoses,not only did the patients suffer from physical impairment,but also various physical and psychiatric comorbidities.Growing evidence have suggested that mental disorders in SLE patients,can lead to various adverse consequences.AIM To explored the features and influencing factors of mental health in patients with SLE and clarifying the correlations between mental health and personality characteristics and perceived social support.The results would provide a basis for psychological intervention in patients with SLE.METHODS The clinical data of 168 patients with SLE admitted at the First Affiliated Hospital of Hainan Medical University between June 2020 and June 2022 were collected.Psychological assessment and correlation analysis were conducted using the Symptom Checklist-90(SCL-90)and Perceived Social Support Scale,and the collected data were compared with the national norms in China.The relevant factors influencing mental health were identified by statistical analysis.A general information questionnaire,the Revised Life Orientation Test,and Short-Form 36-Item Health Survey were employed to assess optimism level and quality of life(QoL),respectively.RESULTS Patients with SLE obtained higher scores for the somatization,depression,anxiety,and phobic anxiety subscales than national norms(P<0.05).A correlation was identified between total social support and total SCL-90 score or each subscale(P<0.05).The factors significantly affecting patients’mental health were hormone dosage and disease activity index(DAI)(P<0.05).The average optimism score of patients with SLE was 14.36±4.42,and 30 cases were in the middle and lower levels.A positive correlation was found between optimism level and QoL scores.CONCLUSION Patients with SLE develop psychological disorders at varying degrees,which are significantly influenced by hormone dosage and DAI.Patients’mental health should be closely monitored during clinical diagnosis and treatment and provided adequate support in establishing positive,healthy thinking and behavior patterns and improving their optimism level and QoL.

Macrophage Activation Syndrome as the Primary Presentation of Pediatric Systemic Lupus Erythematosus: A Case Report and Review of the Literature

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.

BCD020 rituximab bioanalog compared to standard treatment in juvenile systemic lupus erythematosus: The data of 12 months casecontrol study

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.

Outcomes of a 12-month course of early and late rituximab BCD020 biosimilar administration in juvenile systemic lupus erythematosus:A retrospective study

BACKGROUND Juvenile systemic lupus erythematosus(SLE)is a severe,life-threatening disease.However,the role of rituximab in managing juvenile SLE remains undefined,although early biological intervention may improve disease outcomes.AIM To assess the differences in the outcomes of different types of rituximab administration(early and late).METHODS In this retrospective cohort study,the information of 36 children with SLE with administration(LRA)was analyzed.We compared initial disease characteristics at onset,at baseline(start of rituximab),and at the end of the study(EOS)at 12 months,as well as outcomes and treatment characteristics.RESULTS The main differences at baseline were a higher daily median dose of corticosteroids,increased MAS frequency,and a higher Systemic Lupus Erythematosus Disease Activity Index(SLEDAI)in the ERA group.No differences in the main SLE outcomes between groups at the EOS were observed.The part of lupus nephritis patients who achieved remission changed from 44%to 31%in ERA and 32%to 11%in the LRA group.Patients with ERA had a shorter time to achieve low daily corticosteroid dose(≤0.2 mg/kg)at 1.2(0.9;1.4)years compared to 2.8(2.3;4.0)years(P=0.000001)and higher probability to achieve this low dose[hazard ratio(HR)=57.8(95%confidence interval(CI):7.2-463.2),P=0.00001 and remission(SLEDAI=0);HR=37.6(95%CI:4.45-333.3),P=0.00001].No differences in adverse events,including severe adverse events,were observed.CONCLUSION ERA demonstrated a better steroid-sparing effect and a possibility of earlier remission or low disease activity,except for lupus nephritis.Further investigations are required.

Ovarian function in patients with systemic lupus erythematosus:Pathogenesis,drug application and prospective therapies

Systemic lupus erythematosus(SLE)is a chronic autoimmune disease in which multiple organs are damaged that prevails in fertile women.Currently,glucocorticoids and immunosuppressants are widely used to treat SLE patients.However,ovarian dysfunction occurs following the use of these drugs in women with SLE.Here,we summarize recent progress in terms of understanding ovarian injury,the effects of drug application and strategies to improve ovarian function in women with SLE.This review could be helpful to precisely cure SLE in women desiring to have offspring.

Immunotherapies application in active stage of systemic lupus erythematosus in pregnancy:A case report and review of literature

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.

Serum IL-10 from systemic lupus erythematosus patients suppresses the differentiation and function of monocyte-derived dendritic cells

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.

Expression of CD80/CD86 and CTLA-4 mRNA in Peripheral Blood Mononuclear Cells of the Patients with Systemic Lupus Erythematosus

Summary: The role of CD80/CD86 and CTLA-4 in the pathogenesis of systemic lupus erythematosus and their clinical significance was investigated. By using RT-PCR technique, the expression of CD80/CD86 and CTLA-4 mRNA in peripheral blood mononuclear cells (PBMC) were semiquantitatively detected in 32 patients with active SLE. The results showed that the percentage of positive CD86 expression in active SLE was increased significantly as compared with normal controls (90.63 % vs 60.00 %, P<0.01). The mean level of CD86 mRNA expression in active SLE group was markedly higher than in the normal controls (0.6410+0.0174 vs 0.4510+0.0402, P<0.001). Compared with normal controls, the percentage of positive CTLA-4 expression and the mean level of CTLA-4 mRNA expression in active SLE group were both increased significantly (both P<0.01). There was no statistical differences in positive rate of CD80 and the average level of CD80 mRNA between the two groups (both P>0.05). It was concluded that the aberrant expression of CD86 and CTLA-4 might play an important role in the activity and development of SLE.

Pathological significance and regulatory mechanism of lymphotoxin β receptor overexpression in T cells of patients with systemic lupus erythematosus

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.

A Medical Record of Systemic Lupus Erythematosus

Case HistoryPatient Chen, female, 68 years, case number: 9800297; date of hospitalization: May 11, 1998.Chief complaints: Fever accompanied with cough and lassitude for 3 months and skin rash for 7 days.History of present illness: In early Feb. 1998, the patient got fever accompanied with cough, chest distress, and expectoration of sticky sputum. She had been diagnosed at a hospital and later at a clinic as chronic bronchitis and pneumonia, and treated for more than 50 days with antibiotics and medication of some drugs for cough and sputum, but with no complete remission of the symptoms. One week previously, she developed general skin rash mainly in the face, accompanied with itching, high body temperature of 38~39℃, aggravated cough and chest distress, lassitude, poor appetite, slight thirst, absence of bowel movement for 3 days, and yellow brownish urine.

Determination of Serum Interleukin-13 and Nerve Growth Factor in Patients with Systemic Lupus Erythematosus and Clinical Significance

The changes in the levels of serum interleukin-13 (IL-13) and nerve growth factor (NGF) in patients with systemic lupus erythematosus (SLE) and their clinical significance were investigated. Sandwich ELISA was used to determine the levels of serum IL-13 and NGF in 35 SLE patients and 15 normal controls. The results showed that the levels of serum IL-13 (92.69±9.87 pg/ml) and NGF (339.69±25.60 pg/ml) in active SLE patients were significantly higher than those in inactive SLE patients (IL-13, 54.22±9.31 pg/ml; NGF, 300.89±33.51 pg/ml)(P<0.01). The inactive patients also had significantly increased serum levels of IL-13 and NGF as compared with normal controls (IL-13, 35.20±12.70 pg/ml; NGF, 111.40±32.54 pg/ml; P<0.05 and P<0.01, respectively). Spearman correlation analysis revealed that the serum IL-13 levels were correlated with disease activity index of SLE (SLEDAI), ESR and serum levels of C_3 (r= 0.813, 0.504, -0.605, respectively). The serum NGF levels were also correlated with above markers (r=0.442, 0.338, -0.463, respectively). The serum levels of IL-13 and NGF had a positive correlation (r=0.506, P<0.01). It was suggested that IL-13 and NGF might be involved in the pathogenesis of SLE and closely correlated with disease activity.

Expression of Inducible Co-stimulator in Peripheral Blood T Lymphocytes in the Patients with Systemic Lupus Erythematosus

The expression of inducible co-stimulator (ICOS) in peripheral blood T lymphocytes from the patients with systemic lupus erythematosus (SLE) and the role in the pathogenesis of SLE was investigated. By using two-color immunofluorescent staining and flow cytometric assay, the expression levels of ICOS in peripheal blood T lymphocytes from 33 patients with SLE and 16 healthy volunteers were detected. SLE diseases activity index (SLEDAI) of the patients with SLE was used to evaluate the disease activity. The correlation between the ICOS expression and SLEDAI was analyzed among the groups. The results showed that the expression levels of ICOS in T lymphocytes in active SLE group was markedly higher than those in the control and inactive SLE groups (both P<0.01). There was no significant difference in the expression levels of ICOS between the inactive SLE and the control groups (P>0.05). In active SLE and inactive SLE groups, positive linear correlation was found between the levels of the ICOS expression in T lymphocytes and SLEDAI (r=0.711, P=0.001; r=0.561, P=0.03). It was suggested that the expression of ICOS in peripheral blood T lymphocytes from the patients with active SLE was up-regulated and and ICOS might be related to the pathogenesis of SLE.

Glucocorticoid reduction induced chorea in pediatric-onset systemic lupus erythematosus:A case report

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.

Effects of Langchuangjing Granule (狼疮净冲剂) on Apoptotic of CD_4^+T and CD_(19)^+B in Spleen of BXSB Mice with Systemic Lupus Erythematosus

Objective: To study the effect on apoptosis of CD 4 +T、CD 19 +B in spleen of BXSB mice with systemic lupus erythematosus treated with Langchuangjing Granule (LCJG, and to probe into the mechanism of the tr eatment. Methods: The apoptosis was examined by the flow cytometric analysis and immunofluorescence double-staining method. Results: Apoptosis of male BXSB mice speeds up. LCJG can restrain the excessive apoptosis of CD 4 +T and CD 19 +B cells in spleen. Conclusion: LCJG treated systemic lupus erythematosus by restraining the excessive apoptosis of T, B lymphocytes, probably restraining the release of excessive amount of apoptotic DNA fragments, so decreasing abnormal proliferation of B cells and the produce of autoantibodies.

Advances in Diagnosis and Treatment of Neuropsychiatric Systemic Lupus Erythematosus

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.

Gastrointestinal involvement in systemic lupus erythematosus:Insight into pathogenesis, diagnosis and treatment

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.

Dubin-Johnson syndrome with systemic lupus erythematosus: a case report

BACKGROUND: Dubin-Johnson syndrome (DJS) is a rare clinical entity. We describe a case of DJS complicated by systemic lupus erythematosus (SLE). METHODS: A case of congenital hyperbilirubinemia with SLE was evaluated systematically including review of history, physical examination for the stigmata of chronic liver disease, and other investigations. RESULT: Liver biopsy revealed a black liver with preserved architecture suggestive of DJS. CONCLUSIONS: SLE may develop in DJS. The relationship between DJS and SLE in this case is most likely a chance occurrence.

Immunophenotyping of Lymphocyte T and B in the Peripheral Blood of Systemic Lupus Erythematosus

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.