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.

Objective:To explore the value of serum soluble growth stimulation expressed gene 2 (sST2) in predicting prognosis of patients with connective tissue disease-related pulmonary arterial hypertension (CTD-PAH).Methods:This retrospective cohort study enrolled 60 CTD-PAH patients diagnosed by right heart catheterization (RHC) during Jan 2017 and Jul 2022. Baseline and follow-up data collected including the levels of sST2, demographic data, clinical data of CTD and PAH, treatment regimen of CTD and PAH, survival status and the onset time of clinical deterioration events. The primary endpoint was the first occurring of clinical deterioration in 3-years. The t-test or Wilcoxon rank-sum test was used for continuous variables com-parison between different groups, and the chi-square test was used to compare categorical variables. Event-free survival rates were evaluated using Kaplan-Meier analysis and groups comparison were performed by the log-rank test. COX univariate and multivariate proportional hazard models were used to identify independent risk factors for poor prognosis. Results:Compared with the patients with sST2<35 ng/ml, CTD-PAH patients with sST2 ≥ 35 ng/ml showed higher WHO cardiac funcation class (Ⅲ-Ⅳ), mean right atrial pressure, N-terminal probrain na-triuretic peptide(NT-proBNP) and CRP levels[13/34 vs 19/26, χ2=7.19, P=0.007; 4.00(3.00, 6.00)mmHg vs 8.00(4.00, 12.00)mmHg, Z=-2.98, P=0.003; 474.30(135.70, 1 947.50) ng/L vs 2 650.50(485.33, 5 906.50)ng/L, Z=-2.72, P=0.007; 3.24(2.56,7.01)mg/L vs 9.66(3.20, 19.33)mg/L, Z=-2.50, P=0.012]; Kaplan-Meier analysis showed that event-free survival rate in 3 years was significantly lower in patients with sST2 ≥ 35 ng/ml (24.1% vs 84.0%, P=0.001). Multivariate COX regression analysis showed that sST2 was an independent predictor of clinical worsening in patients with CTD-PAH [ HR (95% CI)=1.020 (1.001, 1.039), P=0.035]. Conclu-sion:Serum sST2 is an independent risk factor for clinical deterioration in CTD-PAH patients.

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.

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.

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.

BACKGROUNDTraditional 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.

METHODSWe 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.

RESULTSAll 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.

CONCLUSIONSSix-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.

China ; Double-Blind Method ; Drugs, Chinese Herbal ; adverse effects ; therapeutic use ; Female ; Humans ; Male ; Medicine, Chinese Traditional ; Middle Aged ; Models, Theoretical ; Sjogren's Syndrome ; drug therapy

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.

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.

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.

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.