ObjectiveTo investigate the clinical features of cardiac involvement in polymyositis or dermatomyositis(PM/DM).MethodsAll articles published in Chinese and English were retrieved by searching the electronic resource databases.Theeligible articles that metthe predefined inclusion and exclusion criteria were included and then a systemic review was performed.ResultsTotally 23 articles were enrolled in this study,including 1452 patients.The incidence of cardiac involvement was 27.1％-85％.Heart failure was the most frequent (43.8％-76.9％) symptom.Among the abnormal ECG and UCG,the incidence of conduction abnormalities,left ventricular diastolic dysfunction andhyperkinetic left ventricular contraction 25％-38.5％,27.3％-51.5％ and 11.5％-42.3％ respectively.The pathological findings revealed myocardial inflamma-tion,degenerative changes and necrosis similar to those findings in skeletal muscles.Some patients'condition were improved aftergluco-corticoid stero and immunosuppressant treatment.Thirtypatients (30.9％) died as a direct result of heart disease.ConclusionHeart abnormalities are common in patients with PM/DM,most of them are subclinical,however.The efficacy of gluco corticoid steroid and immunosup-pressant treatment are uncertain.Cardiac involvement is a common cause of death.

ObjectiveTo investigate the relationship between autoimmune thyroid disorders (AITD) and thyroid cancer (TC) in children.Methods Clinical date from 68 patients who underwent thyroidectomy and were histologically conifrmed with TC were retrospectively analyzed from 2000 to 2008. Age, gender, nodular size, lymph node metastasis, and invasive status of thyroid cancer were compared between patients with and without AITD.Results In 68 patients with TC, 45.6% (31/68) patients had concurrent AITD. Compared with those without AITD, a greater female preponderance, older age, smaller tumour size, higher TSH values, higher TG values, shorter survival time, lower disease free survival rate, and higher relapse rate were found in patients with AITD (P<0.05).Conclusions AITD are associated with TC in children. Follow-up is suggested in children with AITD.

Objective To investigate the correlation between serum levels of B cell activating factor (BAFF) and disease activity in the patients with dermatomyositis (DM).Methods Serum BAFF levels of 61 patients with DM and 25 matched healthy controls were measured by ELISA.The results of the two groups were compared using unpaired Mann-Whitney U test and the relevance was analyzed using Spearman correlation analysis.Results Serum levels of BAFF in DM patients were significantly higher compared to healthy controls [(274 7±264 6) pg/ml 与 (832±170) pg/ml,Z=-5.492,P＜0.01].A cross-sectional assessment revealed that serum BAFF levels were positively correlated with global disease activity (r=0.501,P＜0.001),muscle disease activity (r=0.303,P＜0.05),and cutaneous disease activity (r=0.467,P＜0.01).High serum BAFF levels were associated with increased incidence of interstitial lung disease (x2=17.238,P＜0.01).The longitudinal study showed modest correlations between serum BAFF levels and global disease activity (r=0.658,P＜0.01),muscle disease activity (r=0.307,P＜0.05),as well as cutaneous disease activity (r=0.565,P＜0.01).Conclusion Serum levels of BAFF correlate with disease activity in DM patients.The results of this study suggest that BAFF is a serological biomarker for DM disease activity.

Objective This study aims to test the hypothesis that low density granulocytes (LDGs) is involved in the pathogenesis of DM associated-Interstitial lung disease (ILD).Methods Forty eight DM patients (28 with ILD) and 19 age-and sex-matched healthy Chinese volunteers were recruited to this study.LDGs percentage in peripheral blood mononuclear cells (PBMCs) was tested by flow cytometry.Neutrophilrelated genes (LL-37,MPO and MMP-8) expressions in PBMCs were tested by quantitative RT-PCR.Myositis disease activity assessment visual analogue scales (MYOACT) was used to assess the disease activity.Percentages of LDGs were compared in patients with ILD and without by using unpaired t test with Welch's correction,the correlations between LDGs and clinical parameters were further analyzed by linear correlation analysis.The expressions of neutrophil-related mRNA and proteins in PBMCs were compared by using MannWhitney U test.Results LDGs percentage in PBMCs was 7.1-fold higher in DM patients than healthy controls [(9.1±11.5)％ vs (1.3±0.7)％,t=4.664,P＜0.01].LDGs percentage in PBMCs was 2.7-fold higher in DM patients with ILD than DM patients without ILD [(12.3±14.1)％ vs (4.5±2.6)％,t=2.835,P=0.008 3].The mRNA expression level of LL-37,MPO and MMP-8 and LL-37 protein levels in the DM group were significantly higher than those in the control group.LDGs percentage positively correlated with MYOACT lung disease activity scores (r=0.439,P=0.010).Conclusion Percentage of LDGs in PBMCs is significantly increased in DM patients with ILD and positively correlated with MYOACT lung disease activity scores,suggesting that abnormall increasing of LDGs is a potential contributor to the pathogenesis of DM-associated ILD.

Objectives To investigate serum levels of B cell activating factor(BAFF)in Chinese patients with polymyositis(PM)or dermatomyositis(DM),and analyze the correlation of BAFF with autoantibodies and clinical phenotypes.Methods Serum BAFF levels of 28 PM patients and 30 DM patients(study group),and 25 matched healthy controls(control group)were measured by ELISA.Serum anti-Jo-1 antibody levels were also measured by ELISA in all the subjects.The results of the two groups were compared by unpaired t test and the relevance was analyzed by Pearson's correlation analysis.Results Serum levels of BAFF in PM/DM patients were significantly higher compared to healthy controls(P =0.000),but there was no statistically significant difference between the PM and DM patients(P ＞ 0.05).Patients with interstitial lung disease(ILD)had significantly higher serum BAFF level than the patients without ILD(P =0.000)or the controls(P =0.000).Serum BAFF levels of patients with positive antinuclear antibody(ANA)were significantly higher than those with negative ANA(P =0.003).For patients with anti-Jo-1 antibodies,the serum BAFF levels were correlated with the serum concentration of anti-Jo-1 antibodies(r =0.799,P =0.006).Conclusions Serum levels of BAFF are increased in Chinese PM/DM patients.These findings indicate that BAFF may be possibly enrolled in the pathogenesis of PM/DM.Detecting serum BAFF levels could have some implication for the diagnosis and treatment of PM/DM.

ObjectiveTo evaluate the efficacy and safety of hydroxychloroquine(HCQ) in pregnant patients with systemic lupus erythematosus (SLE).Methods Twenty-four pregnant patients with SLE treated with HCQ during pregnancy from May,2006 to February,2011 were studied retrospectively.All babies were followed up during early infancy for growth development.ResultsOf them,22 patients were treated with HCQ throughout the whole pregnancy with no lupus flare occurred in 21 patients (95.4％),while temporary discontinuation of HCQ precipitated a flare of disease in two patients.Three patients ( 12.5％ ) had premature delivery,and pregnancy induced hypertension happened in 3 patients (12.5％ ).No congenital abnormalities occurred and mean follow-up of 26 months ( range 1 - 47 months) revealed no abnormalities in these children.Conclusion Our findings reinforce the safety of HCQ therapy during pregancy and HCQ should probably be maintained throughout the pregancy in patients with SLE.

Objective To investigate the quality of autoantibody testing around the whole country.Methods Laboratories that perform autoantibody testing were recruited by letters or telephone communications.The auto-antibodies examined by the quality-control survey included anti-nuclear antibody (ANA),anti-double-stranded DNA (anti-dsDNA) antibody,anti-extractable nuclear antigens (anti-ENA),antimitochondria antibody (AMA),anti-smooth muscle antibody (ASMA),and anti-citrulline antibody (CCP).Each autoantibody was tested for 3 samples,so 15 samples were tested in total.Sample distribution and data analysis were double-blinded.Qualitative interpretation,staining patterns were evaluated by IIF.The agreement with qualitative interpretation for each specimen was evaluated by ELISA,and Immuno-Blot/Dot-Blot.Results One hundred and eight laboratories participated in this study.The testing methods included indirect immumofluorescence (IIF),immuno-Blot (IB),Dot-Blot (DB),double diffusion method (DID),enzymelinked immunosorbent assay (ELISA),chemo-illuminescent assay,Dot-immunogold filtration assay.The accuracy rates were 82％,83％,95％,96％,86％,respectively for ANA,anti-dsDNA,AMA,ASMA,and anti-CCP antibody.Anti-ENA were further divided into anti-SNP,anti-Sm,anti-SSA,anti-SSB,anti-Scl-70 subgroups,and the accuracy rates were 84％,95％,98％,98％,88％,respectively.The distribution of quantitative values by different laboratories for ANA (by IIF),anti-dsDNA,anti-CCP antibody (by ELISA) varied remarkably.Conclusion Hospitals that enrolled in the survey and the items involved for quality control were increasing year by year.The accurate rates of ANA,anti-dsDNA in this survey were similar to the past national quality control surveys,the quality of AMA/ASMA,anti-CCP antibodies test was better than that of the past surveys,but the quality of anti-ENA antibodies test needs to be improved.

Objective To study the diagnostic value of cartilage oligomeric matrix protein for early cartilage destruction in osteoarthritis and assess its value in the prediction of the disease progression.Methods The osteoarthritis animal models were developed by immobilizing the right knees of 18 rabbits in full extension position using plaster East.Knee joint pathological changes at week 2 and 6 were examined for pathological severity evaluation of osteoarthritis.ELISA sandwich method was used to measure the levels of cartilage oligomeric matrix protein(COMP) in serum before and after modeling(at week 2 and 6 respectively) and immunohistolgy method was used to examine the levels of COMP in knee articular cartilage of osteoarthritis animal models.Correlation analysis was performed to demonstrate the relationship between the levels of COMP in the serum and the pathological severity of osteoarthritis.Pearson's test and t-test were used for correlation analysis.Results ①) Osteoarthritis animal models could be successfully developed by immobilizing the right knees of rabbits in full extension position using plaster east for 2 weeks.Early histopathological changes in the articular cartilage could be observed,At week 6,the typical histopathological characteristics could be seen.②With the extension of modeling time,serum COMP levels persistently increased.The serum COMP levels before modeling,at modeling week 2,week 6 were (3.35±0.20),(3.64±0.18),(3.96±0.44) μg/L respectively,the difference was significant (P＜0.05).③ The level of COMP in the articular cartilage of non-osteoarthritis animal models,models at week 2,week 6 were (2.7±1.8 )％ ,(5.7±0.7)％,(7.6±0.7)％ respectively (P＜0.05 for all).④ The level of COMP in the serum was linearily correlated with the pathological severity of osteoarthritis(r＞0.770 for all,and P＜0.05 for all).Conclusion Levels of COMP in the serum can help to make early diagnosis of osteoarthritis,and elevated COMP level can predict the progression of osteoarthritis.

Objectiye To study the levels of cartilage oligomeric matrixprotein (COMP) and matrix metalloproteinase-3 (MMP-3) in the serum fluid of osteoarthritic rabbit models and their relationships with the severity of pathological changes, so as to investigate their correlation with osteoarthritis(OA). Methods The osteoarthritic animal models were get from immobilizing the right knees of 18 rabbits in full extension using plaster cast. Knee joint pathological changes of 2,6 weeks were examined for pathological severity of OA; ELISA sandwich method was used to measure the levels of COMP and MMP-3 in serum before and after modeling( at 2, 6 weeks respectively); X ray of model keens was also obtained in different period.Correlation analysis was performed to demonstrate the relationship between the levels of COMP, MMP-3 in the serum and the pathological severity of OA. Results ( 1 ) Morphological observations: immobilizing the right knees of rabbits in full extension using plaster cast was a reliable methed for osteoarthritic animal models and the typical histopathologic character was seen; the severity of osteoarthritisgradually increased with time extended. (2) The levels of COMP[(3.64 ±0. 18)μg/L], MMP-3 [(1.99 ±0. 81 ) μg/L]in the serum of 2 weeks osteoarthritic animal models were higher than those before immobilizing with plaster cast [COMP(3.35 ±0. 20) μg/L,MMP-3( 1.61 ±0. 71 ) μg/L]. The levels of COMP[(3.96 ±0. 44) μg/L],MMP-3[(3.44 ±0. 91) μg/L] of 6 weeks were much higher,with a significant difference(P ＜0.05). The levels of COMP, MMP-3 in serum had a linear correlation with the pathological severity of OA (r ＞0. 710,and P ＜ 0. 05 ). Conclusion The levels of COMP and MMP-3 in serum can help to predict and evaluate the progression of OA.

Objective To profile the differentially expressed genes in the muscle of polymyositis (PM) patients.Methods A mRNA microarray analysis was performed to profile mRNAs from 5 treatment-naive PM patients and 5 healthy controls.Gene Ontology and KEGG pathway analyses were applied to delineate the functional roles of the differentially expressed mRNAs.Quantitative real-time PCR analysis was conducted to validate the microarray data.The Student's t-test was used to analyze the statistical significance of the microarray results,and Benjamini-Hochberg FDR was used for multiple-test correction.Results Microarray analysis revealed that a total of 1 905 mRNAs (787 up-regulated and 1 118 down-regulated) were significantly differentially expressed in PM patients compared with the healthy controls (fold change＞2,P＜0.05).Six mRNAs were selected to analyze by quantitative RT-PCR to validate their expression levels and the results were consistent with that of the microarray analysis,and thus provide reliable validation for the microarray results.Gene ontology and KEGG pathway analysis for the differentially expressed mRNAs revealed that these genes were mainly involved in the biological process of infection and cytotoxic effect.In addition,there were some common signaling pathways that shared by PM and other autoimmune diseases.Conclusion There are differences in gene expressions between PM patients and healthy controls.The muscle damage in PM patients may be due to multi gene involvement and multi gene regulation.