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Hemophagocytic syndrome (HPS), which is currently named as hemophagocytic lymphohistiocytosis (HLH), is a hyperinflammatory syndrome characterized by persistent fever, hepatosplenomegaly, pancytopenia and hemophagocytosis found in bone marrow, liver, spleen and lymph nodes due to excessive activation of macrophages and cytotoxic T cells. Macrophage activation syndrome (MAS) is a specific form of HLH induced by autoinflammatory/autoimmune disorders which can be life-threatening and requires multiple disciplines. In order to improve clinicians′ understanding of MAS and standardize the clinical diagnosis and treatment practice of MAS, the rheumatology branch of Chinese Rheumatology Association organized domestic experts to formulate the diagnosis and treatment standard, in order to improve the diagnosis and treatment level of MAS and improve the prognosis of patients.
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Objective:To investigate the role of HIS (hyperinflammatory syndrome) score in predicting the prognosis of anti-melanoma differentiation associated gene 5(MDA5) antibody-positive dermatomyositis (DM) patients with interstitial lung disease (ILD).Methods:A total of 43 patients with anti-MDA5 antibody-positive dermatomyositis and 228 connective tissue disease (CTD) patients with ILD hospitalized in the First Affiliated Hospital of Nanjing Medical University from January 2018 to April 2021 were enrolled into this study. All patients were complicated with ILD and their HIS score were assessed. Non-parametric Mann-Whitney U test, Chi-squared test, Fisher exact probability and receiver operating characteristic (ROC) curve were used for data analysis. Results:The HIS score of 43 patients with anti-MDA5 antibody-positive dermat-omyositis were collected. Among 228 CTD-ILD patients in the control groups, the primary disease consisted of 33(14.5%) anti-synthetase antibody syndrome (ASS), 44(19.3%) rheumatoid arthritis (RA), 65(28.5%) Sj?gren's syndromes (SS), 43 (18.9%) systemic sclerosis (SSc) and 43 (18.9%) systemic lupus erythematosus(SLE). The HIS score of anti-MDA5-positive DM-ILD patients [2(1, 3)] was higher than those in ASS patients [1(0, 2), Z=-2.06, P<0.05] and significantly higher than those in RA-ILD [1(0, 2), Z=-2.87, P<0.01], SS-ILD [0(0,1), Z=-5.78, P<0.01], SSC-ILD [1(0, 1), Z=-3.84, P<0.01] and SLE-ILD [1(0, 2), Z=-3.81, P<0.01]. Comparing HIS score of anti-MDA5-positive DM-ILD patients, the 3-months mortality rate in the low, medium and high groups was 0(0/15), 38.1%(8/21) and 85.7%(6/7). The area under ROC curve (95% CI) was 0.857[(0.747, 0.967), P<0.001] for HIS score in predicting 3-months mortality probability of anti-MDA5-positive DM-ILD. Conclusion:HIS score of anti-MDA5-positive DM-ILD is higher than that in CTD-ILD patients and the baseline level is related to the 3-months mortality rate.
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Immune thrombocytopenia (ITP) is an autoimmune disease mediated by acquired immunity, with platelets decreased and hemorrhage being the primary clinical manifestations. The responses and platelet count levels after early treatment are important factors affecting long-term prognosis, however, current diagnostic methods and disease evaluation approaches are limited, and lack of specific biomarkers. In recent years, various biomolecules have been proposed as potential biomarkers for ITP. This paper reviews the value and advances in ITP-related biomarkers.
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Objective To investigate the clinical characteristics of tuberous sclerosis complex (TSC) with connective tissue disease and to improve the understanding of TSC. Methods The clinical manifestations and laboratory examinations of TSC were analyzed. A case of TSC was reported and the related literatures were reviewed. Results A 50-year-old female presented with multisystem damage such as skin, kidney, respiratory system, central nervous system, hematological system and positive anti-nuclear antibody. Chest CT showed pulmonary lymphangiomyomatosis and cranial MRI showed bilateral ventricular subependyous nodules. Genetic testing revealed a mutation in the TSC2 gene, which was diagnosed as TSC. Conclusion TSC is an autosomal dominant hereditary neurocutaneous syndrome. As the clinical manifestations are diverse, misdiagnosis is often. Rheumatologists should pay attention to it.
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<p><b>BACKGROUND</b>Traditional Chinese medical treatment of primary Sjögren's syndrome has advantages over Western medicine in terms of fewer side effects and improved patient conditions. This study was a multicenter, randomized, double-blind, placebo-controlled clinical trial of the efficacy and safety of ShengJinRunZaoYangXue granules for the treatment of primary Sjögren's syndrome, including the symptoms of dry mouth and dry eye.</p><p><b>METHODS</b>We undertook a 6-week, double-blind, randomized trial involving 240 patients with primary Sjögren's syndrome at five centers in East China. A computer-generated randomization schedule assigned patients at a 2:1 ratio to receive either ShengJinRunZaoYangXue granules or placebo once daily. Patients and investigators were blinded to treatment allocation. The primary endpoints were the salivary flow rate, Schirmer test results, and sugar test results. Intention-to-treat and per-protocol analyses were performed.</p><p><b>RESULTS</b>All 240 patients were randomly allocated to either the treatment group (n = 160, ShengJinRunZaoYangXue granules) or placebo group (n = 80) and were included in the intention-to-treat analysis. After program violation and loss to follow-up, a total of 199 patients were included in the per-protocol analysis. At six week, intention-to-treat and per-protocol analyses of the left-eye Schirmer I test results showed an improved difference of 1.36 mm/5 min (95% CI: 0.03 to 2.69 mm/5 min) and 1.35 mm/5 min (95% CI: 0.04 to 2.73 mm/5 min), respectively, and those of the right-eye Schirmer I test results showed an improved difference of 1.12 mm/5 min (95% CI: 0.02 to 2.22 mm/5 min) and 1.12 mm/5 min (95% CI: -0.02 to 2.27 mm/5 min), respectively. There was no significant difference between the two groups before treatment. After treatment, the between-group and within-group before-and-after paired comparison results were statistically significant (P < 0.05). Intention-to-treat and per-protocol analyses showed an improved salivary flow rate by 0.04 ml/15 min (95% CI: -0.49 to 0.58 ml/15 min) and 0.04 ml/15 min (95% CI: -0.52 to 0.60 ml/15 min), respectively, but the differences were not significant. Intention-to-treat and per-protocol analyses showed that the sugar test results were improved by 1.77 minutes (95% CI: 0.11 to 3.44 minutes) and 1.84 minutes (95% CI: 0.12 to 3.55 minutes), respectively, but the differences were not significant. For the secondary endpoint, intention-to-treat and per-protocol analyses showed significant improvement in the integrated evaluation of treated patients with dry eye and dry mouth after six weeks of treatment. The incidence of adverse events was 15.6% in the treatment group and 10.0% in the placebo group. Most (94%) adverse events were mild to moderate in the two groups, and only two cases of serious adverse events occurred in the treatment group; both were caused by autoimmune liver disease.</p><p><b>CONCLUSIONS</b>Six-week treatment with ShengJinRun ZaoYangXue granules for primary Sjögren's syndrome in this large-scale study improved the symptoms of dry mouth, dry eyes, and low tear flow rate with minimal adverse events.</p>
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Female , Humans , Male , Middle Aged , China , Double-Blind Method , Drugs, Chinese Herbal , Therapeutic Uses , Medicine, Chinese Traditional , Models, Theoretical , Sjogren's Syndrome , Drug TherapyABSTRACT
ObjectiveTo investigate the association of complement C3 with clinical and serological features of patients with systemic lupus erythematosus.MethodsData was collected by the same methods in the past ten years in fifteen hospitals in Jiangsu Province and then data weres summarized for retrospective analysis.Clinical and laboratory data were selected and then analyzed by Chi-square test,Wilcoxon rank sum test and Logistic regression.ResultsOne thousand four hundred and five patients were investigated.One thousand and forty two had low serum complement C3 level.In this case control study,hospitalization age,disease course,admission times,pleurisy,gastrointestinal involvement,general lymphadenopathy/hepatosplenomegaly,white blood cell count, haemoglobin level,platelet count, serum C-reactive protein level,serum albumin level,serum creatinine level,Urine protein quantification,anti-nuclear antibodies (ANA),anti-dsDNAantibodies, anti-SmantibodiesandSLEDAIscore were possible factors associatedwith complement C3 reduction(P＜0.05).Logistic regression analysis showed that CRP (OR=0.396,0.254-0.617,P=0.000),ANA (OR=2.907,1.267-6.670,P=0.012),urine protein level(OR=1.702,1.043-2.779,P=0.033) and SLEDAI score (OR-0.930, 0.886-0.975,P-0.003) were correlated with complement C3 reduction.Conclusion Complement C3 level is valuable for lupus flare assessment.The complement C3 reduction is a risk factor for renal impairment.
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Objective To investigate the prevalence and risk factors of hyperuricemia (HUA) in rural residents of Gaoyou City,Jiangsu Province.Methods A total of 4504 rural residents were investigated in a cross-sectional study.Questionarre survey and physical examination were used for the stndy.Fatsing venous blood samples were collected for biochemical examination.T-test,chi-square test for multiple-group data comparison and logistic regression analysis were used for statistical analysis.Results The prevalence of HUA was 11.9％,15.7％ in male,which was significantly higher than 8.6％ in the female (P＜0.01),the risks of HUA in male was 1.98 times higher than in female.The average value of serum uric acid in this population was higher than.that of female.The prevalence of HUA in female increased with age.Non-conditional logistic regression analysis showed WHR,Cr,Tg,hypertension were the independent risk factors of male patients with HUA.Age,BMI,Cr,Tg,hypertension were independent risk factors of HUA in female.Conclusion The prevalence of HUA can be affected by age and sex.Weight control,keeping blood pressure and blood lipid profiles in normals levels are important for the prevention of HUA.
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Objective To explore the relationship between the impairment of hematological system and disease activity,immunological parameters,and the prognosis of systemic lupus erythematosus (SLE).Methods The clinical and laboratory data of in-patients with SLE in Jiangsu Province were investigated and all patients were hospitalized between 1999-2009.The impairment of hematological system was assessed and the relationship between hematological system damage and disease activity,immunological parameters,mortality rate of patients with SLE were analyzed.Statistic method used was X2 test.Results One thousand nine hundred and fifty eight cases of SLE were included in the study,in which,1836 were female and 122 were male.One thousand five hundred and forty nine (79.1%) patients complicated with hematological system damage,62.3% were anemia,45.5% with leucopenia and 29.4% with thrombocytopenia.There were significant differences in hematological system damage rate among patients with mild activity group,moderate activity group,severe activity group and almost no activity group,compared respectively with almost no activity group.The P values were P=0.01 and P＜0.01 respectively.The incidence of hematological system damage in elevated ESR,low complement C3 level,anti-dsDNA antibody group was higher than that in patients who had normal ESR,complement C3 level and anti-dsDNA group.(P＜0.01).During follow-up,166 patients died,of which the mortality rate(91.6%) in patients had hematological system damage,was obviously higher than those without hematological damage(8.4%)(P＜0.01 ).Among the 166 deceased patients,38.6% died of severe infection,22.9% died ofrenal failure,15.1% died ofnervous system damage,10.2% died of cadiovascular damage and 13.3% died from other causes.Conclusion Hematological system is one of the most commonly involved system in patients with SLE,of which anemia is the most common,and the incidence of leukopenia follows.The impairment of hematological system is closely related to lupus activity.Patients with abnormal immune parameters tend to have secondary hematological system damage.Severe infection is the main cause of death in patients with lupus,followed by nervous system damage and kidney damage.The mortality rate in patients with lupus that complicated hematological system damage is higher than patients who have no hematological system damage.
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Objective To investigate the initial manifestation and disease onset feature of systemic lupus erythematosus(SLE) in the past ten years in fifteen hospitals in Jiangsu Province.Methods Data was collected by the same Methodsin all the participated hospitals and then it was summarized for retrospective analysis.Two groups were compared by chi-square test.Results ① One thousand nine hundred and fifty eight patients were investigated and the male-to-female ratio was 1∶15.0.② One thousand seven hundred and ninty eight patients had clear initial manifestations.The most common initial manifestations were skin and mucosal lesions(769 cases,42.8％ ) and arthritis (697 cases,38.8％ ).The main skin lesion was malar rash (706 cases).Arthritis was found to be more common in female than male.③ All hospitalized patients at their first admission showed multiple organ/system involvement:the most common involvement was skin and mucous membrane (82.3％),hematologic damage (74.0％),in which at least one series of blood cells were involved,arthritis (1156 cases,56.5％ ) much more than myositis (51 cases),proteinuria 1046 cases and hematuria in 385 cases.Renal biopsy pathology showed type Ⅳ glomerulonephritis.Conclusion ① SLE patients are mainly female and the male to female ratio is 1∶15.0.② The most common initial manifestations are skin and mucosal lesions.③ The most commonly involved organ/system are skin and mucous membrane,blood,joint and kidney.The most common pathological changes shown in renal biopsy is type Ⅳ glomerulonephritis.
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Objective To explore the potential role of high levels of adiponectin (AD) in the inflammatory joint of rheumatoid arthritis (RA). Methods ELISA was used to measure the levels of AD, IL-Iβ,IL-6, IL-8, TNF-α, MCP-1 and MMP-9 in the synovial fluids of RA and osteroarthritis (OA), the levels of these cytokines were tested after the synovial fibroblasts (SFLs) were stimulated with AD. Doublelabeling immunohistochemistry was used to analyze the expression of AD in RA synovium. Cytokines were measured by ELISA after SFLs were stimulated with AD. The expression of RANKL was detected by real-time PCR after MH7A were treated with AD and IL-6 ANOVA, Student's t-test, Mann-Whitney U-tese, Spearman's-test were used for statistical analysis. Results High levels of AD in RA synovial fluids were correlated with IL-6 levels. Double labeling immunohistochemistry showed that AD was localized in fibroblasts. MCP-1 and IL-6 were dramatically increased in human synovial fibroblasts following incubation with recombinant AD for 24 h. RANKL mRNA was significantly increased in MH7A after treated with AD and IL-6. Conclusion High levels of AD in the inflammatory joints of RA are likely to contribute to the high expression of IL-6, MCP-1 and RANKL, which may play an important role in the chronic inflammation, osteoclasts activation and bone erosion in RA.
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Objective To establish the rat model of collagen-induced arthritis and study the diagnostic value of MRI for early RA by comparing to pathological changes. Methods Thirty Wistar rats were randomly divided into normal groups and experimental groups.Fifteen rats of the experimental groups were immunized with type Ⅱ collagen and Freund's Adjuvant Incomplete. At the scheduled days, the selected rats of both experimental and normal groups underwent X-ray, 1.5T MR scan plus enhancement and histological analysis. Results Arthritis Index increased on the 14th day after immunization and reached the peak on the 28th day in the experimental group, the positivity of Anti-CⅡ antibody was significantly different from the normal group.The experiemental model was established in 93.33% of all rats. The enhanced MRI showed that 12 (12/15) rats presented with abnormal signs . The sensitivity of MRI was 85.71% and the specificity was 100%. There was no significant correlation could be found between MRI and histological changes X-ray revealed soft tissue swollen in 4 (4/15) rats, which showed that MRI had higher sensitivity in detecting abnormal signs of arthritis than X-ray. Conclusion MRI may be used in the early diagnosis of early RA.
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Objective To study the expression of Adiponectin (AD) and its receptors Adiponectin receptor 1 (Adipo R1) and Adipo R2 in the synovial fluids and the synovium of rheumatoid arthritis (RA). Methods ELISA was used to determine the levels of AD in 23 RA and 23 osteoarthritis (OA) patients. Real-time PCR and Western blot techniques were employed to study the expression of AD, AdipoR1 and AdipoR2 in the synovium of 10 RA and OA patients. Results It was observed that approximately twice more adiponeetin in the synovial fluids of patients with RA than with OA. Adiponectin and AdipoR1, but not AdipoR2 mRNA, were significantly expressed in synovium of RA patients in comparison with OA. Adiponectin and AdipoR1 protein were wuch more expressed in synovium from RA than those from OA. Conclusion High expression of Adiponectin and AdipoRl is likely to contribute to the formation of inflammatory joints in RA.
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Objective Through investigating the effect of mesenchymal stem cells (MSCs) on the expression of yon Willebrand factor (vWF) and soluble thrumbomodulin (sTM) of vascular endothelial cells stimulated by serum of systemic lupus erythematosus (SLE) patients to discuss the repairing effect of MSCs on injured endothelium of SLE patients.Methods When human umbilical vein endothelial cell strain ECV-304 was co-cultivated with serum of SLE patients in vitro for twelve hours in order to induce endothelial cells injury,then MSCs were seeded for three days.Enzyme linked immunosorbent assay (ELISA) was used to detect the expression of vWF and sTM in the supernatant.Results When ECV-304 was stimulated by serum of SLE patients,the expression of vWF and sTM in the supernatant was significantly higher than that in the groups not stimulated by serum of SLE patients;When MSCs were seeded ,the expression of vWF in the Lupus serum stimulated group in which MSCs were seeded was significantly lower than that in the lupus serum stimulated group in which MSCs were not seeded,but the expression of sTM between the lupus serum stimulated group which MSCs were seeded and the lupus serum stimulated group in which MSCs were not seeded was not significantly deviatied.Conclusion Serum of lupus patients at active stage can cause injure vascular endothelial cells.MSCs can downregulate the expression of vWF of vascular endothelial cells.These suggest it may participate in the repairing of injured vascular endothelium of SLE patients.