Objective The aim of this study was to describe the clinical and pathological features of primary biliary cirrhosis(PBC) and their correlation.Methods Liver biopsy specimens were obtained through percutaneous needle puncture from twenty four patients with PBC who had not been diagnosed or treated before.These samples were fixed in formaldehyde and embedded in paraffin for routine histological examination.Pathologic stages based on Ludwig criteria,fibrosis,portal and periportal inflammation,lymphocytic periportal piecemeal necrosis,ductular proliferation,intralobular hepatocyte necrosis,the degree of ductopenia and relevant laboratory results were recorded.Statistics method used was x2 or t-test,Mann-whitmey U nonperametric test and Pearson's or Spearman's correlation analysis.Results The pathological stages,degree of fibrosis were positively correlated with total bilirubin (TBIL) level,total bile acid (TBA),cholesterol (CHO),IgG levels,and were negatively correlated with serum albumin(ALB) level(r=-0.527,P=0.030; r=-0.503,P=0.039) ,percentage of eosinophilic cells (EOS) ( r=-0.554,P=0.021; r=-0.502,P=0.040).Lymphocytic periportal piecemeal necrosis was positively correlated with alkaline phosp-hatase (ALP),TBIL,DBIL,TBA,and also tumor necrosis factor-αt (TNF-αα) levels(r=0.617,P=0.006).Conclusion TBIL,DBIL,TBA,CHO,IgG and ALB,EOS are good surrogate markers for disease sever ity and reversibility of PBC,while ALP,TNF-Cα,TBIL,DBlL,TBA can be used as markers for disease activity.

Objective To detect the expression of T helper 17 (Th17) cells in the peripheral blood of patient with ankylosing spondylitis (AS),and discuss its role in thc pathogenesis of AS.Methods Twenty AS patients and fifteen healthy controls were enrolled in the study.Th17 (IL-17),Th1 (IFN-γ),Th2 (IL-4)cells and HLA-B27 of their peripheral blood were analyzed by flow cytometry,at the same time,the levels of ESR and CRP were also measured in order to analyze thc relation of Th17 and HLA-B27,ESR as well as the CRP level.The statistical analysis was carried out with single-sample t-test and Speraman's correlation test.Results The level of Th17 cells was significantly higher in the perpipheral blood of AS patients[(2.6±0.8 )%] than those in healthy controls[ (1.1±0.4)% ] (P＜0.01).The level of Th 1 cells was significantly lower in the peripheral blood of AS patients[(3.9±0.8)%] than those in healthy controls[(5.1±1.3)%] (P＜0.01)and the level of Th2 cells was not different in the peripheral blood of AS patients[(4.1±1.6)%] when compared to healthy controls[ (3.1±1.4)% ] (P＞0.05).Th 17 cells was not significantly correlated with the percentage and mean fluorescent intensity of HLA-B27,ESR,CRP(P＞0.05); but there was a tendency that increased expression level of Th17 cells was associated with elevated percentage and mean fluorescent intensity of HLA-B27.Conclusion The level of Th1 cells is decreased,but Th17 is increased in the peripheral blood of AS patients.Th cells are imbalance in AS,patients.The change of Th17 cells may be an important part of the pathogenesis of AS.

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.

Objective The aims of this study were to compare the clinical and laboratory responses to ursodeoxycholic acid (UDCA) monotherapy with the combination therapy of UDCA plus prednisolone/azathioprine in primary biliary cirrhosis(PBC),and to investigate the risk factors affecting the therapeutic responses.Methods Eighty-two patients with untreated PBC were divided randomly into three groups.Group U (28 patients) received UDCA alone,group UP(27 patients) received UDCA and pr ednisolone,while group UA (27 patients ) received UDCA and azathioprine.The clinical and laboratory data were recorded after treated for 3,6 and 12 months.Repeated measures ANOVA and COX regression model were used for statistical analysis.Results Fatigue and pruritus were improved only in group UP(P=0.015 and P=0.037 respectively).Alanine aminotransferase(ALT),aspartate aminotransferase(AST),alkaline phosphatase(ALP),gamma-glutamyl transferase (GGT),total bilirubin (TBIL),direct bilirubin (DBIL) and IgM in the 3 groups were decreased (P＜0.05),while there was no statistical significant difference between the three groups (P＞0.05).The patients with disease progression had higher Mayo risk score (MRS) (P=0.018) and prolonged prothrombin time (PT)(P=0.042).In group UP,side-effect associated with glucocorticosteroids occurred in eight patients while there was no side-effect in group U.High baseline levels of ALP、GGT and CHO were risk factors for biochemical remission(P=0.015).The increase of DBIL,TBIL,total bile acid(TBA) and PT did not contribute to the prediction of biochemical remission ( P=0.075 ).Conclusion There are no differences among the three groups in the improvement of hepatic biochemical data and IgM.The combination therapy of UDCA with prednisolone could relieve fatigue and itching.The disease of patients with higher Mayo risk score and prolonged PT tend to progress.High baseline levels of ALP,GGT and CHO are risk factors for biochemical remission.High baseline levels of TBIL,DBIL,TBA and PT could not predict biochemical remission.The incidence of adverse effect is lowest when treated with UDCA alone.

Objective To detect the serum level of tartrate-resistant acid phosphatase 5b (TRACP5b) in patients with rheumatoid arthritis (RA) before and after infliximab treatment and analyze the relevance between TRACP-5b and activity indexes of RA.The effect of different medicines on bony erosion in RA and its possible mechanism were explored.Methods Patients were divided into two groups:16 patients were treated with inffiximab for 24 weeks (group 1 ),25 patients were treated with MTX for 24 weeks (group 2).The core indicators of RA activity were evaluated.Ranked test,grouped design and matched t test was used to examine the quantity data between groups,while numerate data was analyzed with qui-square test.Correlation between data was tested by Spearmen's and Pearson's tests.RestltsThe levels of TRACP-5b in patients with X-ray stagesⅠ ,Ⅱ ,Ⅲ ,Ⅳ were elevated at the baseline.The levels of TRACP-5b in phase Ⅲ and Ⅳ were [(3.34±1.07) U/L] and[(4.05±0.25) U/L] respectively,higher (P＜0.05) than those in phase Ⅰ[(1.69±0.48) U/L].At week 0,week 12,and week 24,the serum levels of TRACP-5b in group 1 were(3.07±1.32),(2.72±1.18),(2.16±1.09) U/L respectively,while the levels in week 12,and 24 were significantly lower than those of week 0(P＜0.05).A significant decrease of serum TRACP-5b level in group 1[(2.16±1.09 ) U/L]when compared to group two[ (3.05±0.93) U/L] after 24 weeks.TRACP=5b level was positively correlated with the course of disease (r=0.313,P=0.043 ),HAQ(r=0.443,P=0.007).Conclusion Infliximab can reduce TRACP-5b level in RA and inhibit inflammatory bone loss.TRACP-5b can be used to evaluate the efficacy of biological agents in treating RA.

Objective The aim of this study was to assess the status of protease-activated receptor-2(PAR-2) expression on the peripheral blood immune cells including dendritic cells(DC) of Wegener's granulo matosis(WG) patients.Methods Flow cytometry was used to analyze the expression of PAR-2 protein on peripheral blood immune cells,DC and DC-like monocytes of healthy controls (HC),inactive,active and different medicine-treated WG patients.Two-tail Mann Whitney non-parametric test was used for statistical analysis.Results There was no difference of PAR-2 expression on PMN,monocytes,lymphocytes and DC between WG patients and HC.However,PAR-2 expression on CD14+CD16+ (t=3.823,P=0.004) and CD64+CD16+ (t=2.652,P=0.024) DC-like monocytes was much higher in WG patients compared with HC.In active WG,expression of PAR-2 was up-regulated on the cell surface of PMN (t=2.690,P=0.013 ),monocytes (t=1.688,P=0.047),lymphocytes(t=1.742,P=0.038),BDCA3+DC(t=2.582,P=0.016),CD11c+DC(t=1.828,P=0.044),CD14+CD16+ (t=3.419,P=0.002)and CD64+CD16+ (t=3.494,P=0.005) DC-like monocytes when compared with inactive WG.PAR-2 expression on these cells was similar between cyclophosphamide (Cyc) and Methotrexate (MTX) treated WG patients.Conclusion PAR-2 expression on immune cells,DC and DC-like monocytes correlates with WG occurrence and development.There is no difference in the effects on PAR-2 expression between various medications.

Objective To explore the effect of the JAK/STAT signal pathway on the apoptosis-related gene in the synovial tissue of rat rheumatoid arthritis (RA).Methods Fifty rat models of collagen-induced arthritis,whose arthritis index was more than 2,were divided into the model group,the low dose of AG490 group,the medium dose of AG490 group and the high dose of AG490 group.In addition,6 rats were treated intraperitoneal injection.Then,the arthritis index and the change of apoptosis-related genes were compared.Multiple-sample average was analyzed by single-factor x2 test and LSD-t or Tamhane's T 2 test were used for two-two comparison.Results The arthritis index of the model group increased evidently,and the apoptosis inhibitor Bcl-2,Bcl-xl gene and protein expression was up-regulated,which was significantly different when compared with that of the control group(0.931±0.035 vs 0.351±0.024,0.920±0.037 vs 0.271±0.029,0.322±0.047 vs 0.230±0.031 ).The expression of apoptosis promoting factor Bax wasslightly up-regulated.The blockage of JAK/STAT pathway cotld down-regulate the expression levels of the gene and protein of survivins Bcl-2 and Bcl-xl,and up-regulate the gene and protein expressions of Bax.Conclusion In the process of RA development,apoptosis inhibitor Bcl-2,Bcl-xl gene and protein expression is up-regulated.JAK/STAT signal transduction pathway regulates the apoptosis process.

Objective To investigate the difference and treatment strategy of malignancy-associated dermatomyositis and other para-neoplastic neurological syndromes (PNS).Methods The clinical characteristics of a patient with malignancy-associated dermatomyositis and poly radiculoneuropathy was reported and the relevant literature was reviewed.Results Patients with dermatomyositis had increased risk of malignancies,and should routinely screened.Dermatomyositis and polyradiculoneuropathy was clinically similar,but could rarely be seen in the same malignant patient.Malignancy -associated dermatomyositis and PNS had similar pathogenesis.The treatment strategy of both was similar.Malignancy specific treatment should be initiated and immune suppressive agents should be prescribed concurrently.Conclusion Rheumatolgists should aware the association between dermatomyositis and potential underlying malignancies.Multiple para-neoplastic syndromes could be seen in the same patient,but the diagnosis should be considered as one.

Objective To explore the potential association between HLA-DRB1 Alleles and systemic scleroderma (SSc) of the Zhuang Nationality in Guangxi region. Methods Polymerase chain reaction-special sequence primers (PCR-SSP) was used to study the HLA-DRB1 alleles in 58 patients with SSc and 50 healthy controls of the Zhuang Nationalty in Guangxi Province. Comparisons between groups were performed with χ2 test or exact probabilities. Results Sixteen HLA-DRB1 alleles were discovered from the specimens,including 14 in the SSc specimens, and 16 in the control specimens. Among them,the allele frequencies of HLA-DRB1 * 1301 (7.760%, OR=9.000, χ2=4.341, P=0.037), HLA-DRB1 * 1305 (11.207%, OR=3.322,χ2=4.206, P=0.040) and DRB1 * 15 (26.724%, OR=2.679, χ2=6.038, P=0.014) were significantly higher in SSc patients than those of the controls (respectively for 1.000%, 4.000%, 15.000%). Conclusion Our data suggest that the HLA-DRB1 * 1301, HLA-DRB1 * 1305 and HLA-DRB1 * 15 may be the susceptible genes of SSc in Zhuang nationality population.