TWo cases of systemic lupus erythernatosus with complement C_4 deficiency which were certified by xperiment were reported in this paper.The results of long follow-uP observation suggested thattwo patients ersistently exist lower complement C_4 in serum.One of these two patients havingcombined deficiency of C_3 and _ uffered from pulmonary infection repeatedly.Forthmore,webriefly analysed the relationship between complements nd ystemic lupus erythematosus.

Objective To study the effect of dehydroepiandrosterone (DHEA) on the immune system by measuring the apoptosis and expression of Fas/FasL mRNA of thymocytes. Methods The cell viability of thymocytes was determined by MTT assay. Thymocyte apoptosis was detected by agrese gel electrophoresis. The expression of Fas and Fas-L was analyzed by a semi-quantitative reverse transcriptase-polymerase chain reaction (RT-PCR) method. Results DHEA decreased the cell viability of thymocytes and induced thymocytes apoptosis and increased the levels of mRNA of Fas/FasL. Conclusion DHEA can induce apoptosis of thymocytes through Fas/Fas-L pathway. It suggests that DHEA may play a role in certain autoimmune diseases related to apoptosis disturbance.

ObjectiveTo investigate the effect of bone marrow-derived mesenchymal stem cells (MSCs) on collagen-induced arthritis(CIA) and the possible mechanisms. MethodsMSCs from C57BL/6 mice bone marrow were isolated and expanded. Three DBA-I mice were as normal control group (Sodium Chloride injected on day 0 and 21) after primary immunization. After the bovine collagen Ⅱ induced-CIA models were established, fifteen male mice were randomly divided into 3 groups according to treatment regi-mens: CIA control group(0.2 ml Sodium Chloride injected through caudal vein on day 0 and 21), prevention group(1×10+6 cells MSC on day 0) and treatment group. (1×10+6 cells MSC on day 21 after primary immun-ization). The observed parameters included arthritis index (Al), articular pathology changes, levels of serum TNF-α, IL-1β detected hy ELISA. In addition, the percentage of CD4+CD25+Foxp3+Treg cells(Tregs) in CD4+ T cells of spleen or lymphoid node from mice were also analyzed by flow cytometry(FCM). Results①The Al and articular pathological score of the prevention group and treatment group were lower than the CIA control group(P<0.05). ②The levels of serum TNF-α and IL-1β of normal control, prevention group and treatment group were significantly decreased compared with CIA control group(P<0.05), and positively correlated with Al or articular pathological score (P<0.05). ③The percentage of Tregs from spleen and lymph nodes was significantly increased in MSCs treated mice(P<0.05). Conclusion MSCs transplantation is effective for CIA and suppression of TNF-α, IL-1β and up-regulation of Tregs by MSCs may be the possible mechanism in CIA.

0 05).Conclusion C kit level may be more closely related to the clinical parameters in SLE patients,which may reflect the clinical status of SLE patients.

Objective To assess the effects of protease 3(PR3)and protease-activated receptor (PAR)-2-activator on the maturation and functions of peripheral blood dendritic cell(DC)-like monocytes.Methods Density gradient centrifugation was used to isolate peripheral blood mononuclear cells(PBMC)from Wegener's granulomatosis(WG)patients and healthy controls(HC).PBMC were stimulated by LPS,human PR3,trypsin,PAR-2-agonist peptide (PAR-2-AP),LPS+PR3 or LPS+trypsin for 24 h.Flow cytometry was used to analyze the expression of PAR-2,CD80,CD83,HLA-DR on stimulated DC-like monocytes-CD14+CD16high monocytes.ELISA kit was used to test the concentration of IL-6 in the culture supernants.Mann-Whitney non-parameteric test was used fur statistical analysis.Resuits No effect of PR3,trypsin and PAR-2-AP on the expression of PAR-2,CD80,CD83,HLA-DR of DC-like monoeytes was found.LPS could significantly induce PAR-2 expression in HC[from(5.8±1.5)%to(24.5±4.5)%,P=0.002]and the expression of CD80,CD83,HLA-DR in HC and WG;PR3,trypsin,PAR-2-AP and LPS could all stimulate the secretion of IL-6.Conclusion PR3 and PAR-2 pathway-activators can not promote PAR-2expression and maturation of DC-tike monocytes,but they can induce the secretion of IL-6.

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 investigate the effect of estrogen on dendritic cells (DCs) and its role in disease progression by comparing the effects of 17β-estradiol (E2) on spleen DCs in systemic lupus erythe matosus (SLE) murine modeI-(NZBxNZW) F1 (NZB/w F1 ) female mice before and after the disease onset.Methods Anti-CD11c antibody labeled magnetic bead was used to purify spleen DCs.Flow cytometry was utilized to detect the co-stimulatory molecules and intracellular cytokines of DCs.Mixed lymphocytes reaction (MLR) was used to measure the stimulatory activity of DCs to T iymphocytes.And semi-quantitative RT-PCR was employed to check the expression of estrogen receptor (ER).Results E2 could modulate the expression of surface molecule CD40,the production of cytokines IL-6,IL-10,IL-12 and TNFa,and the stimulatory a bility of spleen DCs in SLE model mice.Tamoxifen (TAM) could antagonize E2 effects and E2 could affect the estrogen receptor (ERα) level of DCs.These changes of DCs varied with age.Conclusion E2 may be in volved in the pathogenesis of SLE by modulating DCs by binding ERa.The effects of E2 vary in different stages of SLE progression.

Objective To investigate the mechanisms of estrogen in the pathogenesis of systemic lupus erythematosus(SLE),we compared the effects of 17β-estradiol(E2)on bone marrow(BM)-derived dendritic cells (BMDCs)in(NZBxNZW)F1(NZB/w F1)female mice befbre and after the disease onset.Methods Immature,BMDCs were differentiated from BM monocytes cultured with rmGM-CSF,rmlL-4,E2 and estrogen receptor (ER)modulator-tamoxifen(TAM)for 7 days.Then immature DCs were stimulated by LPS for 24h to get mature BMDCs.Flow cytometry was utilized to detect the production of costimulatory molecule CD40 and intracellular IL-6,IL-10,IL-12 and TNFα of DCs.Mixed lymphocytes reaction was used to measure the stim-ulatory activity of DCs on spleen T lymphocytes.Results E2 mainly increased the expression of CD40 on im-mature BMDCs but reduced its expression on mature BMDCs.E2 enhanced the stimulatory activity of immature BMDCs,but weakened the activity of mature BMDCs.E2 decreased the production of cytokines of BMDCs in young mice,but increased them in old mice.Conclusion E2 modulates the functions of BMDCs of lupus model mice by binding with ER.The modulation varies depending on disease progression and cell maturation status.

Objective Analyzing polymyositis (PM) and dermatomyositis (DM) cases that had respiratory failure at the initial presentation in order to get a better understanding of their clinical features. Methods Eight PM or DM patients with respiratory failure as their first symptom were included in this study. Their clinical manifestation, laboratory and radiology changes and prognosis were analyzed retrospectively. Results In 8 patients, respiratory failure occurred before skin and muscle symptom for about two weeks to 3 month. Pulmonary interstitial disease was revealed by chest X ray and CT. Pulse methylprednisonlone was used combined with anti-infection and ameliorating respiratory function treatment. Five of the patients became deteriorated and 3 died of respiratory failure despite of the treatment, while the other 3 improved but one died of respiratory failure 6 months later. Conclusion About 5%~30% patients with PM and DM have lung damage. Pulmonary changes may occur before skin and muscle symptoms in 4% patients. Such patients have abrupt onsets and their disease progresses quickly with high mortality despite of corticosteroids therapy.