Objective To assess the possible differences of the clinical features and renal pathology between children and adults with Henoch Schoelein purpura (HSP),and the contribution of clinical and renal biopsy parameters to predict the disease outcome.Methods A retrospective study was performed in 156 patients with HSP.Patients younger than 16 years at disease onset were considered children,and those aged 16 years or over were considered adults.Results The male was more prevalent in both adults and children HSP groups (M∶F=1 5∶1).The previous drug treatment,and special food intake were more frequent among the adults ( P =0 03 and 0 009,respectively).Renal involvement,increased ESR and serum IgA levels were more frequent in adults,but vomiting and joint pain were more in children.The frequencies of previous upper respiratory tract infection (URTI),fever,abdominal pain,melena and nephrotic syndrome were similar in both groups.Multivariate analysis showed that age at the disease onset,URTI and abdominal pain were predictive for renal involvement (RR=7 8,4 1 and 4 6,respectively).There were no differences of the renal pathologic types between two groups.However,lesions other than glomeruli including tubular and interstitial involvement were more frequent in adults.The outcome was better in children after a mean follow up of 6 2 years.Renal disease was the main clinical manifestation in non complete remission (NCR) patients.The proteinuria and the other area lesions out of glomeruli predict the decreased remission rate (RR=5 3 and 6 7,respectively).Conclusion These results indicate that HSP is more serious and nephritis is more frequent in adults.Proteinuria and lesions other than glomeruli are the higher risk factors of NCR.

Objective The purpose of this study is to analyse the expression of tumor necrosis factor (TNF)-related apoptosis inducing ligand(TRAIL)and its receptors in muscle tissue from patients with idiopathic inflammatory myopathies(ⅡM)and investigate the possible role of TRAIL system in the Dathogenesis of IIM.Methods TRAIL and its receptors DR4,DR5,DCRl and DCR2 were detected in the musck biopsy tissue from 36 patients with ⅡM(13 polymyositis,23 dermatomyositis)and 9 heMthy controls bv using immunohistochemistry. Results The expression of TRAIL and its receptors was f10und in muscle tissue samples from the IIM patients and healthy controls.The expression of TRAIL,DR4 and DCR2 in the muscle tissue from the IIM patients was significantly higher than those from healthy controls(all P values< 0.05).The expression of TRAIL,DCRl and DR4 was also detected in the infiltrating lymphocytes in endomysium and in the interstitial tissue around blood vessels.Conclusion The diversity in expression of TRAIL and its receptors between patients with IIM and healthy controls suggests the hypothesis of a crucial role of TRAIL in the pathogenesis and the pathology of IIM.

ObjectiveTo systematically review and evaluate the predictive factors of interstitial lung disease(ILD) in patients with polymyositis and dermatomyositis (PM/DM), and provide evidence for early clinical diagnosis. MethodsThe predictive factors of PM/DM-related ILD from 23 published papers were analyzed by Meta-analysis. Analysis of heterogeneity and sensitivity were performed and pooled odds ratio (OR) with 95％ confidence interval(95％CI) were calculated using either fixed or random effects models by Rev-Man 4.2 software. Stata 10.0 software was used to identify publication bias. ResultsMeta-analysis was performed to analyze seven factors including anti-Jo-1 antibody, fever, arthritis/arthralgia, Gottron's sign,antinuclear antibody(ANA), and dysphagia Raynaud's phenomenon. Factors correlated with ILD in patients with PM/DM and their OR values and 95％CI were as follows: the presence of anti-Jo-1 antibody 6.94 (4.74～10.16), fever 4.90(3.82～6.29), arthritis/arthralgia 3.93(3.21～4.80), Gottron's sign 2.52(1.24～5.14), and the presence of ANA 1.59( 1.02～2.47 ). Raynaud's phenomenon and dysphagia were not correlated with ILD in patients with PM/DM with OR values and 95％CI as 1.40 (0.97～2.01), and 1.21(0.94～1.56) respectively.ConclusionThe presence of anti-Jo-1 antibody, fever, arthritis/arthralgia, Gottron's sign and ANA are major predictive factors for ILD in patients with PM/DM.

Objective:To investigate the expressions of CD4+ CD25+ T cells and IL-10 in the peripheral blood of patients with systemic lupus erythematosus(SLE) and their clinical significance.Methods:Thirty SLE patients(seventeen active and thirteen remissive) and twenty normal controls were enrolled in the study. Flow-cytometric assay was employed for detection of CD4+ CD25+ T cells,and double antibody sandwich ELISA was applied to detect IL-10 in sera from SLE patients and normal controls.Results:The levels of CD4+ T cells in active and remissive SLE were significant lower than normal controls; the positive rate of CD4+ CD25+ T cells in both active and remissive SLE was higher than that in normal controls; the levels of IL-10 in active stage of SLE were significant higher than in remissive stage of SLE or in normal controls. No correlation was found among the levels of CD4+ CD25+ T cells or IL-10 in SLE with SLEDAI scores, anti-DNA and C3 level.Conclusion:CD4+ CD25+ T cells in the peripheral blood of patients with SLE are the marker for activation of T-cells, and aberrant IL-10 production was related with onset of SLE.

3 on biopsy contributed significantly to the prognostic value of RSR,and the presence of tubular atrophy was associated with decreased both SR and RSR.Conclusion These results indicate that early diagnosis is significantly associated with increased SR and RSR.Cr and 24UP are independent indicators of prognosis in clinical respect.Chronic renal histological features and renal artery lesion serve an additional important role in the assessment of prognosis in patients with LN.

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.

Objective To detect the prevalence of myositis-specific autoantibodies (MSAs) and myositis-associated autoantibodies (MAAs) in patients with polymyositis (PM) and dermatomyositis (DM), and analyse the correlation between MSAs and the clinical features and prognosis of PM/DM. Methods Serum samples of 31 PM and DM patients were screened for MSAs (including anti Jo-1, anti Mi-2, anti PL-7, anti PL-12 antibodies) and MAAs (including anti Ku, anti PM-Scl antibodies) by immunoblotting test. Results Serum MSAs/MAAs were detected in 18 out of 31 PM/DM patients (58%). MSAs were present in 12 patients (39%). The most frequently encountered MSAs was anti-Jo-1 autoantibody (29%), followed by anti-Mi-2 (7%), anti-PL-7 (3%), and anti-PL-12 (3%). MAAs were present in 10 patients (32%), including anti-Ku-72 (16%), anti-Ku-86 (23%) and anti-PM/Scl (7%). Notably, anti-Jo-1 antibody was closely associated with interstitial lung disease (ILD) and arthritis/arthralgia compared with the anti-Jo-1 antibody negative patients (P

Objective To study the expression of major histocompatibility complex(MHC)on muscle biopsy specimens of idiopathic inflammatory myopathies(IIM),and assess diagnostic value of MHC in IIM. Methods Forty-five patients with IIM(19 polymyositis and 26 dermatomyositis)were selected for this study.Thirty healthy subjects were included as controls.Immunohistochemical staining was applied to identify the expression of HLA-A/B/C and HLA-DR on muscle biopsy-specimens in polymyositis (PM)/deFmatomyosiris (DM) patients and healthy controls.Results HIA-A/B/C antigens were expressed in muscle fibers in 18 out of 19 PM patients(95%),24 out of 26 DM patients(92%) and 3 out 0f 30 healthy controls(10%) respectively.The positive expression rate of HLA-DR in PM,DM patients and healthy controls were 84%, 81%,13%,respectively.The expressions of HLA-A/B/C and HLA-DR were significantly increased in PM and DM patients than those in healthy controls(both P<0.05),but no significant differences were found between PM and DM groups(both P>0.05).No significant correlations were demonstrated between the over-expression of HLA-A/B/C or HLA-DR and the extent of inflammatory infiltrations,muscle damage or clinical features in PM/DM groups (all P values>0.05).Conclusion MHC-Ⅰ and MHC-Ⅱover-expression in muscle fibers are the early events in PM and DM,and may occur in the absence of lymphocyte infihration and muscle damage.Immunostaining for MHC-Ⅰ and MHC-Ⅱcan be used as a routine test in the diagnosis of PM and DM.

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.

ObjectiveTo determine the serum level of anti-aminoacyl-tRNA synthetase (ARS)antibody in patients with polymyositis (PM) and dermatomyositis (DM) and to investigate the value of anti-ARS antibody for diagnosing interstitial lung diseases(ILD) in patients with PM/DM compared with anti-Jo-1 antibody.MethodsSerum anti-ARS antibody concentrations were measured by ELISA in 109 adult PM/DM patients,20 patients with SLE,20 patients with RA and 50 healthy controls.T test,Mann-Whitney U test,chi-square test or Fisher exact test were used to compare the sensitivity and specificity for diagnosing ILD in PM/DM patients between anti-ARS antibody and anti-Jo-1 antibody.Moreover,McNemar test was employed to analyze the correlation between the clinical features and anti-MDA5 antibody in PM/DM patients.Results The serum positive rate of anti-ARS antibody was 37.9％,7.8％,10％,0 and 0 in PM/DM patients with ILD and without ILD,patients with SLE and RA and healthy controls,respectively.Serum anti-ARS antibody levels and positive rate in the PM/DM patients with ILD were significantly higher when compared with PM/DM patients without ILD,patients with SLE and RA and healthy controls (X2=-13.5,5.45,10.57,15.17; P＜0.01 ).Anti-ARS antibody presented a significantly higher sensitivity for diagnosing ILD in patients with PM/DM compared to anti-Jo-1 antibody (P＜0.01).The rate of fever and ILD were significantly higher in anti-ARS positive group than anti-ARS negative group(X2=12.55,13.53; P＜0.05),while heliotrope rash and shawl sign occurred more often in anti-ARS negative group(X2=5.7,5.8; P＜0.05).Additionally,follow-up study showed that the serum anti-ARS antibody were all negative in nine patients who died of PM/DM with ILD (P＜0.05).ConclusionSerum anti-ARS antibody is a stronger predictor for early diagnosis of PM/DM with ILD compared to anti-Jo-1 antibody.The detection of anti-ARS antibody can be widely applied to clinical practice.