ObjectiveTo further understand the clinic manifestations of childhood primary Sjogren's Syndrome(pSS) and enhance early diagnosis. MethodsFive cases of pSS from Renji Hospital, Shanghai, were reported and their clinical features were analysoed. And literatures from Medline database and Weipu database were reviewed and discussed. Results①Childhood pSS had various clinic presentations that were non-specific and sicca symptoms were absent or occur late in most cases. ② The most common presentations were recurrent parotiditis and cutaneous manifestations with various locations and forms. ③ American-European Criteria for SS were not suitable for the diagnosis of childhood pSS. ConclusionRecurrent parotiditis and cutaneous manifestations in children can be used as clues for the diagnosis of childhood pSS but needs to be further confirmed by the positive results of salivary gland biopsy and autoantibodies examination, particularly SSA/SSB.

ObjectiveTo investigate the association between serum level of osteopotin(OPN) and disease activity of rheumatoid arthritis (RA) patients, and explore the importance of OPN in the pathogenesis of interstitial lung disease (ILD) in RA. MethodsSixty-five RA patients and 20 healthy controls were pros-pectively enrolled. RA patients were divided into active group(n=43) and inactive group(n=22), and ILD groups (n=24) and non-ILD group (n=41). Enzyme-linked immunosorbent assay (ELISA) was used to detect the serum levels of OPN in patients with RA and healthy controls, and the relationship between OPN and other clinical and laboratory findings were analyzed.Results① Serum OPN tended to be significantly higher in RA patients (median, 18.0 ng/ml) than in the healthy controls (median, 14.3 ng/ml), P<0.01; ②The serum level of OPN in RA patients showed a significant positive correlation with the course of disease, numbers of tender joints , ESR and CRP, but no positive relationship was found in number of swollen joints; ③ The serum level of OPN was significantly higher in RA-ILD patients(median, 20.0 ng/ml) than that in non-lLD (median, 17.0 ng/ml, P<0.05). And there was remarkable negative correlation between the concentration of serum OPN and the value of PaO2, but no association was found with pulmonary function %VC and %DLCO. ④ Compared with the non-ILD group, the ILD group had more active disease in terms of tender joint counts and swollen joint counts, ESR, CRP(P<0.01) and the serum titer of RF-IgM,(P<0.05). ConclusionOPN plays a role in the pathogenesis of RA and is related to the disease activity. It may serve as an active disease inflammatory marker of RA . OPN may be involved in the pathogenesis of RA related ILD and is associated with the severity of pulmonary damage.

Objective To study the methylation of CpG motifs and expression of lymphocyte function-associated antigen-1 (LFA-1) mRNA in patients with systemic lupus erythematosus (SLE), and evaluate their associations. Methods The peripheral blood lymphocytes were collected from 26 patients with SLE and 17 normal individuals. The methylation of CpG motifs was detected by the 5-methylcytosine antibody and flow eytometry, and the expression of LFA-1 mRNA was analyzed by RT-PCR. Results Methylation of CpG motifs in patients with SLE was lower than the control subjects (P<0.05), and a negative correlation existed between methylation of CpG motifs and SLEDAI (P<0.05). While expression of LFA-1 mRNA in patients with SLE was higher than that in the control group (P<0.05), and a positive correlation could be detected between the expression of LFA-1 mRNA and SLEDAI (P<0.05). There was a negative correlation between methylation of CpG motifs and expression of LFA-1 mRNA (P<0.05) in patients with SLE. Conclusion Hypomethylation of CpG motifs does exist in patients with SI,E, and is correlated with high expression of LFA-1, therefore, epige-netics plays an important role in the pathogenesis of SLE.

Objective To analyze the outcomes and prognostic factors associated with the death of systemic lupus erythematosus (SLE) patients admitted to the intensive care unit (ICU). Methods During June 1996 to June 2007, all SLE patients admitted to the ICU were included. Patients were excluded if the diagnosis of SLE was established at or after ICU admission. A multivariate logistic regression model was applied using variables that were associated with death in the univariate analysis. Results A total of 101 patients meeting the criteria were included. The mortality rate was 48.6%. The most common causes of admission was lung disorder with acute respiratory distress syndrome (ARDS). Multivariate logistic regression analysis suggested that SLICC/ACR DI>7.7 (OR=6.87), APACHE Ⅲ≥21 (OR=29.8), lung disorders with ARDS (OR =55.81 ), septic shock (OR =32.22 ), intracranial haemorrhage (OR =57.35 ), hypocytopenia (OR = 5.89), mean equivalent prednisone dose>25 mg/d (OR=7.65) and prolonged tracheal intubation (OR=5.98) were signi-ficantly associated with death. Whereas sex, age, SLEDAI >27, gastrointestinal bleeding, the cumulative dosage of CTX higher than 1.0 g, pulse intravenous methylprednisolone therapy were not associated with death. Conclusion The mortality rate of critically ill SLE patients in ICU is very high. SLICC/ACR DI> 7.7, APACHE Ⅲ≥21, lung disorders with ARDS, septic shock, intracraniai haemorrhage, average prednisone equivalent dosage higher than 25mg/d and prolonged tracheal intubation (longer than 4 days) are negative prognostic factors in SLE patients admitted to the ICU.

Objective To investigate the relationship between the single nucleotide polymorphism of aminoimidazole carbexamide ribonucleotide transformylase gene and the efficacy and toxicity of methotrexate treatment in rheumatoid arthritis. Methods Total of 359 patients with RA were divided into mono-therapy with MTX group, combination therapy with other DMARDs group and other DMARDs combination with no MTX treatment group. The clinical and laboratory measurements were evaluated before therapy and 12, 24 weeks after therapy. Efficacy (evaluated by ACR20) and side effects of the drugs were also assessed. Real-time fluorescent quantitative PCR was conducted to test ATIC 347C/G mutation in RA patients and 340 healthy controls. Results There was no statistical significant difference in 347 CC, CG, GG between RA patients and healthy controls. In the MTX mono-therapy group (n=107), 72% (n=77) there was no statistical significant difference in 347CC, CG, GG between patients with good response and patients without efficacy. 32.7%(n=35) of these patients experienced adverse drug reactions. The ATIC G allele carriers (22.4%) experienced a greater frequency of side effects than the CC carriers (OR=2.672, 95%CI, 1.27～5.59, P<0.05). In MTX combined with other DMARDs group (n=128) and other DMARDs combination without MTX group (n=90), the polymorphism in the ATIC gene was not associated with good clinical response and adverse events (P>0.05). Conclusion There is no statistical significant difference between RA and healthy controls in the ATIC347 gene. Polymorphism in the ATIC gene is not associated with clinical response to MTX treatment, but the ATIC347 G allele is associated with MTX toxicity. It maybe used to predict the adverse drug reactions of patients who take MTX.

Objective To evaluate the efficacy of medical supervised aerobic exercise on physical activity, quality of life and psychological status in patients with systemic lupus erythematosus (SLE). Methods SLE patients who fulfilled ACR criteria were recruited and divided into 2 groups: exercise group (n=24) and control group (n=25). The patients in the exercise group were supervised to have aerobic exercises. The intensiveness of exercise was determined by 20%-40% of maximum heart rate reservation. Visual analog scale (VAS), SLE disease activity index (SLEDAI) score, physical working capacity (PWC170), SF-36 and profile of mood states (POMS) of the two groups were used to evaluate the changes at the baseline, 1 month, 3 months and 6 months after this study. Results The 2 groups were homogeneous and comparable in disease activity at baseline. 1, 3 and 6 months after the study, the VAS, PWC170, POMS and SF -36 of SLE patients were improved in certain degrees in both groups, while the improvement of VAS (P<0.05), PWCITO (P< 0.01 ) and social function of SF-36 (P<0.05) of exercise group were significantly more evident than those of the control group in 6 months after study without any impact on disease condition. There was a high negative correlation between VAS and 5 categories of POMS (r=-6.26~-0.393, P<0.01 ) and a more relevant positive association between VAS and 2 categories of POMS (r=0.534~0.611, P<0.01). Conclusion The data demonstrate that the supervised aerobic exercise can ameliorate physical capacity, improve quality of life and psychological and emotional status in the state SLE patients without aggravating disease per se.

Objective To investigate the clinical-pathological features, treatment and prognosis of thrombotic thrombocytopenic purpura in patients with lupus nephritis (LN). Methods A retrospective ana-lysis was carried out based on the clinical-pathological data for the treatment and prognosis of eight patients with LN related TIP. All patients had thrombocytopenia and hemolytic anemia, neurological symptoms and renal dysfunction. Six patients had fever. Results All 8 patients had sudden-onset of rapid progressive glomeurlonephritis (RPGN), one patient with continuous gross hematuria, the pathological features of these patients revealed TMA lesions. Immune suppressive therapy was initiated and blood purification therapy were applied in seven patients. Three cases had plasmapheresis and (or) immunoabsorption. One case was lost during follow-up, the other seven patients were followed with period at one year. One patient died, three patients went into peritoneal dialysis in which one of them was changed to hemodialysis finally. The other three patients had stable renal function. Conclusion The LN patients with TTP had severe clinical-patho-logical changes, rapid progressive and poor outcome, so we should diagnose and treat these patients as early as possible.

Objective To investigate the effects of methylprednisolone pulse therapy on the expression of phosphorylated signal transducer and activator of transcription 1 (STATI) and DNA-binding activity of STATI in T cells in patients with severe systemic lupus erythematosus (SLE). Methods Six patients were included. Patients were given 0.5～1 g of methylprednisolone on 3 consecutive days. Western Blotting was conducted to explore the phosphorylated STATI expression and electrophoretic mobility shift assays (EMSA) were carried out to detect the DNA-biding activity of STATI. Results Methylprednisolone pulse therapy decreased phosphorylated STATI expression of T cells from patients with severe SLE. The expression of phosphorylated STATI decreased to about 30% 72 h after the methylprednisolone pulse therapy started (t=2.858, P<0.05). Methylprednisolone pulse therapy down-regulated DNA-biding activity of STATI of T cells in patients with severe SLE. The STATI DNA-biding activity was inhibited to about 40% 72 h after methy-Iprednisolone pulse, therapy started (t=3.058, P<0.05). Conclusion Phosphorylated STATI expression and DNA-binding activity of T cells is markedly decreased in patients after methylprednisolone pulse therapy, suggesting that inhibition of STATI signaling contributes to the clinical efficacy of this agent.

Objective This study has explored the role of antibody against annexin A2 in patients with antiphospholipid syndrome (APS) and systemic lupus erythematosus (SLE). Methods Using purified recombinant annexin A2, IgG anti-annexin A2 antibody was measured by ELISA in 101 APS patients, 41 SLE patients with thrombosis, 124 SLE patients without thrombosis and 120 healthy controls. Results The positive rate of IgG anti-annexin A2 antibody in APS patients and SLE patients with thrombosis was 21.8%, 26.8%, respectively, they were all significantly higher than in SLE patients without thrombosis (6.5%). IgG anti-annexin A2 antibody was associated with thrombosis and/or pregnancy morbidity (P<0.01). Conclusion Anti-annexin A2 antibody is associated with thrombosis and/or pregnancy mnrbidity. It suggests that anti-annexin A2 antibody may be helpful in identifying in some potential AIRS.

Objective This study was designed to investigate the expression changes of osteopro-tegerin (OPG), tartrate-resistant acid phosphatase (TRAP) and vascular endothelial growth factor (VEGF) mRNA in collagen induced arthritis(CIA) rats. Methods After CIA was induced in Sprague-Dawley rats, the experimental animals were treated with PBS or rAAV-EGFP or rAAV-hOPG (100 μl/day) intra-articular injection for 10 days. Messenger RNAs (mRNAs) were obtained from CIA synovium 40days after first immun-ization. Reverse transcriptase-polymerase chain reactions (RT-PCR) were carried out to detect the mRNA encoding OPG, TRAP, VEGF and β-actin, which acted as inner control. The genes detected clearly by RT-PCR were quantified using real-time PCR. Results The expression of all genes was confirmed by specific single bands in RT-PCR. Real-time PCR showed that the expression levels of TRAP and VEGF were increased, whereas those of OPG mRNA were decreased in CIA group compared with normal controls. The intra-articular gene transduction markedly increased the gene copies of OPG by 128.21% (P<0.01). The expression change of OPG in synovium also caused the decrease of the expression levels of TRAP and VEGF by 58.79% (P<0.01)and 17.85% (P>0.05) respectively, however, the expression change of VEGF was not statistically significant. Conclusion OPG gene mediated by rAAV can be successfully tranfered to knee joint synovium in vivo. The results of this study suggest that gene transfer using rAAV-OPG may be a feasible and effective therapeutic approach to treat or prevent joint destruction in inflammatory arthritis.