Associations with Organ Involvement and Autoantibodies in Systemic Sclerosis: Results from the Canadian Scleroderma Research Group (CSRG)

Objective: Serum from SSc patients was analyzed centrally to determine ANA patterns and extractable nuclear antigens (ENAs) between lcSSc and dcSSc and associations with organ involvement. Methods: 1145 SSc patients had ANA and ENA analyzed by indirect immunofluorescence on HEp-2 substrate at a screening serum dilution of 1/160. Most ENA antibodies [Sm. U1-RNP, Ro52, SS-A/Ro60, topoisomeraseI (Topo1), SS-B/La, chromatin, ribosomal P and Jo1] were measured by laser bead immunoassay;and RNA polymerase III (RNAP) by ELISA. Results: ANA was positive in 95% (same in lcSSc, and dcSSc). Centromere pattern was present in 34%, speckled 22%, nucleolar 18%, homogeneous and speckled (H&S) 16%, multiple nuclear dots 6%. Anti-centromere Ab (ACA) occurred in 46% of lcSSc and 11% of dcSSc (P = 0.0001). ENAs that differed between lcSSc and dcSSc subsets were Topo1 (OR 2.4, P = 0.0001) and RNAP (OR 5.6, P 0.0001) more common in dcSSc. Overall, 15% had positive Topo1;usually with a H&S pattern (67%);Topo1 was associated with ILD on CXR (OR 2.3;95% CI 1.5 - 3.5) and HRCT (OR 3.8;95% CI 1.8 - 8.2). RNAP occurred in 18.5% (35.4% in dcSSc vs. 8.9% in lcSSc). Scleroderma renal crisis (SRC) was 13 times more likely if RNAP positive;P = 0.0001. ACA was only weakly associated with sPAP > 50 mmHg (OR 1.8;95%CI 1.1 - 3.0). Conclusion: ANA homogeneous pattern alone is rare in SSc;ACA was significantly more common in lcSSc. Many ENAs are equal in lcSSc and dcSSc except RNAP and Topo1. RNAP has the highest OR of SRC. Topo1 is less strongly associated with ILD. Abstract word count: 249, Body word count 1246, Figures 2, Tables 2. Key Messages: 1) 95% of SSc has a positive ANA and ANA patterns in SSc include centromere, nucleolar, and homogeneous and speckled together;2) Most ENAs are equal in both dcSSc and lcSSc except anti RNA polymerase III and topoisomerase I;3) RNA polymerase III has the highest association (odds ratio) with scleroderma renal crisis, topoisomerase I is associated with interstitial lung disease;whereas anticentromere was not associated with elevated pulmonary arterial pressures on echocardiogram.

Predictors of a Cerebrovascular Accident in a Population of Systemic Sclerosis Patients Followed at a Large Academic Center with a Dedicated Scleroderma Center

Aim: The aim of this study is to describe the clinical characteristics of patients with a diagnosis of systemic sclerosis who later suffer a stroke and to identify associations for this relationship. Background: Prior studies have showed an increased risk of cardiovascular disease among patients with chronic inflammatory disorders, with chronic inflammation leading to atherosclerosis believed to be the culprit. Systemic sclerosis (SSc) is a chronic inflammatory disease characterized by diffuse fibrosis of the skin and internal organs. Previous studies have suggested a possible link between systemic sclerosis and macrovascular complications such as stroke. Methods: This is a retrospective chart review of patients treated within the University of Pennsylvania Health System from October 2015 to April 2019 with a diagnosis of SSc. Using ICD10 codes, we identified a cohort of SSc patients who suffered a stroke. Information regarding demographics and stroke risk factors were gathered from the charts of patients with a diagnosis of both SSc plus stroke and compared to a control group of randomly selected patients with SSc who never suffered a stroke. Continuous variables were conveyed using a mean plus a standard deviation. A two-sample t-test was used to compare the two groups of patients. Qualitative variables were compared using a two-tailed Fisher’s exact test. Results: Based on a large cohort of SSc patients (n = 2080) followed between October 2015 and April 2019, we identified 36 SSc patients who developed a subsequent stroke (1.7% of cohort). When looking at risk factors for stroke in SSc patients, we identified hypertension and atrial fibrillation to be associated with the diagnosis of stroke in such patients. Specifically, 28 of the 36 patients with both SSc and stroke also had a diagnosis of hypertension while in the control group, only 17 of 36 patients had hypertension. Atrial fibrillation was seen in 9 of 36 patients with both SSc and CVA while it was seen in only 2 of 36 patients in the control group. Conclusions: This case control study demonstrated that the presence of hypertension and atrial fibrillation had a statistically significant association with the diagnosis of CVA in patients with SSc.

Anaemia in a Patient with Diffuse Systemic Scleroderma

We hereby present a case of anaemia in a 73 years old patient with known past medical history of diffuse systemic scleroderma, who presented with acute onset of dizziness and haemetemesis. Blood tests revealed sudden drop of haemoglobin and an urgent gastroscopy revealed gastric antral vascular ectasia (GAVE) or “watermelon stomach”. GAVE is a rare but well recognised cause of acute bleeding in systemic scleroderma patients and should be kept as a differential diagnosis in the work up of anaemia in these patients.

Estimating Benefits from Immunesuppressive Treatment in Diffuse Cutaneous Systemic Sclerosis: Data from the Canadian Scleroderma Research Group

Objective: To determine the efficacy of immunesuppressive treatment over 1 year in early diffuse cutaneous systemic sclerosis (dcSSc). Methods: dcSSc patients with less than 3 years disease duration and at least one year of data enrolled in the CSRG database were included. Regression analyses for achieving at least minimal important differences (MID) for 5 outcomes over one year were done to determine baseline predictors of change and if immunesuppressive treatment yielded the attainment of the MID. Results: 124 patients (mean age 52.3 years;79.2% female) were included. Variables associated with MID at one year were often the baseline variable and for some outcomes, age, sex, smoking, restrictive lung disease and treatment type. Treatment with immunesuppressive was not found to be associated with achieving MIDs in multivariate analyses. Conclusion: Treatment was associated with achieving a MID change at 1 year using univariate statistics, but not in multivariate models. These observational data do not support improvement with immunesuppressives over one year but there could be confounding or biases comparing with those prescribed immunesuppressives vs. those who don’t.

Juvenile Systemic Sclerosis: About 9 Cases

Scleroderma is a rare disease with two primary forms: localized scleroderma (LS) and systemic sclerosis (SSc). Both are chronic conditions that can manifest in various patterns (subtypes) and are linked to extracutaneous involvement in pediatric patients. Juvenile SSc poses a higher risk of morbidity and mortality, with patients facing life-threatening complications such as lung, heart, and visceral organ fibrosis, and vasculopathy. In contrast, mortality is extremely rare in juvenile LS, but patients are susceptible to significant morbidity, leading to severe disfigurement and functional impairment. Treatment for scleroderma aims to control inflammation and address specific issues. An early diagnosis significantly enhances the overall outcome. This study conducts a retrospective descriptive analysis aiming to document the clinical manifestations, management approaches, and outcomes of systemic sclerosis in a cohort of nine children receiving treatment for juvenile systemic sclerosis at Pediatric B department of Mohammed VI University, Hospital Center in Marrakech, Morocco.

Present and future of new systemic therapies for early and intermediate stages of hepatocellular carcinoma

Hepatocellular carcinoma(HCC)is a high mortality neoplasm which usually appears on a cirrhotic liver.The therapeutic arsenal and subsequent prognostic outlook are intrinsically linked to the HCC stage at diagnosis.Notwithstanding the current deployment of treatments with curative intent(liver resection/local ablation and liver transplantation)in early and intermediate stages,a high rate of HCC recurrence persists,underscoring a pivotal clinical challenge.Emergent systemic therapies(ST),particularly immunotherapy,have demonstrate promising outcomes in terms of increase overall survival,but they are currently bound to the advanced stage of HCC.This review provides a comprehensive analysis of the literature,encompassing studies up to March 10,2024,evaluating the impact of novel ST in the early and intermediate HCC stages,specially focusing on the findings of neoadjuvant and adjuvant regimens,aimed at increasing significantly overall survival and recurrence-free survival after a treatment with curative intent.We also investigate the potential role of ST in enhancing the downstaging rate for the intermediate-stage HCC initially deemed ineligible for treatment with curative intent.Finally,we critically discuss about the current relevance of the results of these studies and the encouraging future implications of ST in the treatment schedules of early and intermediate HCC stages.

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.

Exploring the impacts of major events on the systemic risk of the international energy market

This study examines the systemic risk caused by major events in the international energy market(IEM)and proposes a management strategy to mitigate it. Using the tail-event driven network(TENET)method, this study constructed a tail-risk spillover network(TRSN) of IEM and simulated the dynamic spillover tail-risk process through the cascading failure mechanism. The study found that renewable energy markets contributed more to systemic risk during the Paris Agreement and the COVID-19pandemic, while fossil energy markets played a larger role during the Russia-Ukraine conflict. This study identifies systemically important markets(SM) and critical tail-risk spillover paths as potential sources of systemic risk. The research confirms that cutting off the IEM risk spillover path can greatly reduce systemic risk and the influence of SM. This study offers insights into the management of systemic risk in IEM and provides policy recommendations to reduce the impact of shock events.

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.

Preoperative systemic inflammatory response index as a prognostic marker for distal cholangiocarcinoma after pancreatoduodenectomy

BACKGROUND The relationship between preoperative inflammation status and tumorigenesis as well as tumor progression is widely acknowledged.AIM To assess the prognostic significance of preoperative inflammatory biomarkers in patients with distal cholangiocarcinoma(dCCA)who underwent pancreat-oduodenectomy(PD).METHODS This single-center study included 216 patients with dCCA after PD between January 1,2011,and December 31,2022.The individuals were categorized into two sets based on their systemic inflammatory response index(SIRI)levels:A low SIRI group(SIRI<1.5,n=123)and a high SIRI group(SIRI≥1.5,n=93).Inflam-matory biomarkers were evaluated for predictive accuracy using receiver operating characteristic curves.Both univariate and multivariate Cox proportional hazards analyses were performed to estimate SIRI for overall survival(OS)and recurrence-free survival(RFS).RESULTS The study included a total of 216 patients,with 58.3%being male and a mean age of 65.6±9.6 years.123 patients were in the low SIRI group and 93 were in the high SIRI group after PD for dCCA.SIRI had an area under the curve value of 0.674 for diagnosing dCCA,showing better performance than other inflammatory biomarkers.Multivariate analysis indicated that having a SIRI greater than 1.5 independently increased the risk of dCCA following PD,leading to lower OS[hazard ratios(HR)=1.868,P=0.006]and RFS(HR=0.949,P<0.001).Additionally,survival analysis indicated a significantly better prognosis for patients in the low SIRI group(P<0.001).CONCLUSION It is determined that a high SIRI before surgery is a significant risk factor for dCCA after PD.

Participant Heterogeneity of Systemic Scleroderma Interventional Trials Worldwide

Objective:Heterogeneity of participants in clinical trials distorts intervention efficacy.However,factors associated with participant heterogeneity in randomized clinical trials(RCTs)focusing on systemic sclerosis(SSc)are not clear.We conducted this systematic review to establish normative standards for future research and help develop management guidelines.Methods:Three databases and 4 registries were searched to identify characteristics of SSc RCTs across different countries.Risk of bias was assessed by the Cochrane Collaboration’s tool and logistic regression was performed to calculate crude and adjusted odds ratios.Results:In total,261 trials met our inclusion criteria.The quality of SSc RCTs worldwide was relatively poor,with no trend of improvement in recent years,and only 12.2%were ranked as having a low risk of bias.Trials with a low risk of bias as well as single-center,single-country,or open-label trials tended to have better participant adherence than trials with a high risk of bias and multiple-center,multiple-country,or double-blind trials.Interestingly,trial registration and primary outcome definition contributed to high withdrawal.National income was also relevant;participant adherence in high-income countries,but not in upper-and lower-middle-income countries,was significantly altered by different variables.Conclusion:Overall,the risk of bias,national income,and trial design may lead to participant heterogeneity of SSc RCTs and ultimately confound the general clinical utility of the results.Trials with a rigorous design and transparent conduction protocol are crucial for obtaining unbiased data that can serve as a reference and for maintaining the fundamental repeatability of SSc RCTs.

Research Progress of Systemic Immune Inflammatory Index in Prostate Cancer

Prostate cancer has gradually risen to become the second most common cancer threatening men’s health, and prostate-specific antigen (PSA), as the main screening indicator for prostate cancer, has the defects of low specificity and insufficient diagnostic efficacy. As a novel inflammatory index based on neutrophil, lymphocyte and platelet counts, the systemic immune-inflammation index (SII) has recently become a more powerful biomarker for predicting the occurrence and progression of various malignancies. SII reflects the systemic inflammatory response of prostate cancer patients in a more balanced manner, and has higher predictive value than neutrophil-to-lymphocyte ratio (NLR) and platelet-to-lymphocyte ratio (PLR). High SII values are often associated with cancer progression and poor prognosis. This article reviews the research progress of SII in prostate cancer, in order to provide guidance for clinical practice.

Predictive value of systemic immunity index for sepsis in low-medium risk community-acquired pneumonia

Objective:To assess the predictive value of systemic immune inflammation index(SII)for sepsis in low-and medium-risk community-acquired pneumonia.Methods:A total of 589 elderly patients with low-and medium-risk community-acquired pneumonia admitted to the Emergency Department of the Second Affiliated Hospital of Hainan Medical University from January 2020 to January 2023 were included as the research subjects,and the general information and laboratory test results of the patients were collected,and the optimal cut-off value of continuous variables for predicting sepsis in elderly patients with low-and medium-risk community-acquired pneumonia was determined by plotting the receiver work characteristic(ROC)curve,which was converted into dichotomous variables and univariate and multivariate logistic Regression analysis of the influencing factors of sepsis in elderly patients with low-and medium-risk community-acquired pneumonia.Based on this,a nomogram model is constructed to predict the risk of sepsis.The differentiation,consistency and accuracy of the model were verified by calibration curve and subject operating characteristic(ROC)curve,and the clinical utility of the model was determined by decision curve analysis.Results:A total of 589 elderly patients with low-and intermediate-risk community-acquired pneumonia were included in this study,of which 96(16.30%)developed sepsis.There were significant differences in age,diabetes mellitus and chronic obstructive pulmonary disease,Lac,PCT,SII and other indexes between sepsis and non-sepsis groups(P<0.05).Logistics regression analysis showed that age,diabetes mellitus and chronic obstructive pulmonary disease,Lac,and SII were independent risk factors for sepsis in elderly patients with low-and medium-risk community-acquired pneumonia.The nomogram prediction model was used to verify the results,and the AUC was 0.826(95%CI:0.780-0.872),and the calibration curve tended to the ideal curve with good accuracy.The decision curve shows that when the threshold of the model is between 0.10~0.78,the model has the advantage of clinical benefit.Conclusion:The nomogram prediction model constructed based on SII to predict sepsis in elderly patients with low-and medium-risk community-acquired pneumonia has good accuracy,which can predict the occurrence of sepsis early,help early identification of high-risk groups and timely intervention,and thus improve the prognosis of patients.

Assessment of systemic immune-inflammatory index and other inflammatory parameters in predicting mortality in patients with acute cholecystitis:A retrospective observational study

Objective:To investigate the effectiveness of the systemic immune-inflammatory(SII)index and other inflammatory parameters in predicting mortality among patients with acute cholecystitis(AC).Methods:279 Patients presented to the emergency department with abdominal pain and diagnosis of AC between September 2021 and September 2023 were included in the study.Demographic data,laboratory parameters,clinical follow-ups,and outcomes of the patients were recorded.Results:The mean age of the patients was(55.0±16.3)years and 36.6%were male.63.8%Had gallbladder/choledochal stones and 49.5%underwent surgery.The mortality rate was 6.1%.Advanced age(P=0.170)and prolonged hospitalization(P=0.011)were statistically significant risk factors for mortality.Decreased lymphocyte count(P=0.020)and increased C-reactive protein(CRP)levels(P=0.033)were found to be risk factors for mortality.According to the mortality predictor ROC analysis results,the cut-off for SII index was 3138(AUC=0.817,sensitivity=70.5%,specificity=84.7%),the cut-off for neutrophil count was 15.28×10^(3)/mm^(3)(AUC=0.761,sensitivity=52.9%,specificity=95.0%),the cut-off for leukocyte count was 19.0×10^(3)/mm^(3)(AUC=0.714,sensitivity=52.9%,specificity=98.0%),cut-off for CRP was 74.55(AUC=0.758,sensitivity=70.5%,specificity=79.0%),cut-off for aspartate transaminase(AST)was 33.0 IU/L(AUC=0.658,sensitivity=82.3%,specificity=50.3%).Conclusions:The SII index may be a good predictor of mortality with high sensitivity and specificity.Elevated levels of neutrophils,leukocytes,CRP,and AST are other inflammatory parameters that can be used to predict mortality associated with AC.

Effect of a systemic intervention combined with a psychological intervention in stroke patients with oropharyngeal dysfunction

BACKGROUND Stroke frequently results in oropharyngeal dysfunction(OD),leading to difficulties in swallowing and eating,as well as triggering negative emotions,malnutrition,and aspiration pneumonia,which can be detrimental to patients.However,routine nursing interventions often fail to address these issues adequately.Systemic and psychological interventions can improve dysphagia symptoms,relieve negative emotions,and improve quality of life.However,there are few clinical reports of systemic interventions combined with psychological interventions for stroke patients with OD.AIM To explore the effects of combining systemic and psychological interventions in stroke patients with OD.METHODS This retrospective study included 90 stroke patients with OD,admitted to the Second Affiliated Hospital of Qiqihar Medical College(January 2022–December 2023),who were divided into two groups:regular and coalition.Swallowing function grading(using a water swallow test),swallowing function[using the standardized swallowing assessment(SSA)],negative emotions[using the selfrating anxiety scale(SAS)and self-rating depression scale(SDS)],and quality of life(SWAL-QOL)were compared between groups before and after the intervention;aspiration pneumonia incidence was recorded.RESULTS Post-intervention,the coalition group had a greater number of patients with grade 1 swallowing function compared to the regular group,while the number of patients with grade 5 swallowing function was lower than that in the regular group(P<0.05).Post-intervention,the SSA,SAS,and SDS scores of both groups decreased,with a more significant decrease observed in the coalition group(P<0.05).Additionally,the total SWAL-QOL score in both groups increased,with a more significant increase observed in the coalition group(P<0.05).During the intervention period,the total incidence of aspiration and aspiration pneumonia in the coalition group was lower than that in the control group(4.44%vs 20.00%;P<0.05).CONCLUSION Systemic intervention combined with psychological intervention can improve dysphagia symptoms,alleviate negative emotions,enhance quality of life,and reduce the incidence of aspiration pneumonia in patients with OD.

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.

Optimizing care for gastric cancer with overt bleeding:Is systemic therapy a valid option?

Gastric cancer(GC)and gastroesophageal junction cancer(GEJC)represent a significant burden globally,with complications such as overt bleeding(OB)further exacerbating patient outcomes.A recent study by Yao et al evaluated the effectiveness and safety of systematic treatment in GC/GEJC patients presenting with OB.Using propensity score matching,the study balanced the comparison groups to investigate overall survival and treatment-related adverse events.The study's findings emphasize that systematic therapy can be safe and effective and contribute to the ongoing debate about the management of advanced GC/GEJC with OB,highlighting the complexities of treatment decisions in these high-risk patients.

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.

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.

Portal vein pulsatility:An important sonographic tool assessment of systemic congestion for critical ill patients

In this editorial we comment on the article by Kuwahara et al,published in the recent issue of the World Journal of Cardiology.In this interesting paper,the authors showed a correlation between portal vein pulsatility ratio,examined by bedside ultrasonography,and prognosis of hospitalized patients with acute heart failure.Systemic congestion is being notoriously underdetected in the acutely ill population with conventional methods like clinical examination,biomarkers,central venous pressure estimation and X-rays.However,congestion should be a key therapeutic target due to its deleterious effects to end organ function and subsequently patient prognosis.Doppler flow assessment of the abdominal veins is gaining popularity worldwide,as a valuable tool in estimating comprehensively congestion and giving a further insight into hemodynamics and patient management.