Objective:To analyze the frequency and characteristics of polymyositis (PM) in idiopathic inflammatory myopathy (IIM), and to investigate whether PM is over-diagnosed.Methods:Patients diagnosed as IIM according to the Bohan & Peter criteria of IIM hospitalized in the Department of Rheumatology of China-Japan Friendship Hospital from 2008 to 2019 were involved in the study. Definite PM (dPM) was defined as typical clinical and pathological features including elevated creatine kinase (CK) level, muscle weakness and muscle biopsy findings with endomysial CD8 + T cell infiltration and expression of MHC-1 on sarcolemma. Meanwhile, dermatomyositis (DM), anti-synthase syndrome(ASS), immune-mediated necrotic myopathy(IMNM), sporadic inclusion body myositis(sIBM) and other myopathies were excluded according to the new classification criteria of IIM subtypes respectively. Statistical analysis was performed using SPSS software 24.0. The Kruskal-Wallis test and χ2 test were used to compare the clinical characteristics between the dPM group and other IIM subtypes. Results:A total of 1 259 patients with IIM including 1 015 (80.6%) DM and 244(19.4%) PM were enrolled in this study. According to the strict definition of PM criteria, only 0.5% of patients (6/1 259) in IIM could be diagnosed as dPM. Most PM patients were IMNM and ASS according to the new IIM subtypes criteria, of which 48.0% (117/244) were IMNM and 32.0% (78/244) were ASS. 66.7%(4/6) of dPM patients were women. One complicated with RA, and one was dPM overlaped with systemic sclerosis. All of them had muscle weakness, mild elevation of CK level [611(391,1 451) U/L], and were myositis-specific autoantibodies negative. Except one dPM patients who did not receive immunoregulatory therapy due to chronic obstructive pulmonary disease, the others were administrated with low or medium dose prednisone combined with or without immunosuppressive agents. After a median follow-up of (38±26) months, the muscle strength of dPM patients were improved.Conclusion:dPM is a very rare clinical subtype of IIM. PM is an over-diagnosed entity in clinical practice. Patients with dPM have mild symptoms and good outcome.

Objective:To identify dermatopathological features of patients with dermatomyositis (DM) and analyze its correlation with cutaneous diseases activity.Methods:The clinical data and skin biopsies of 48 patients were collected. The relevance was analyzed using Spearman's correlation analysis. The two groups were compared using Chi-square test or Fisher's exact test. Multi-factors line regression model was established to analyze the relationship between cutaneous disease activity and dermatopathological features.Results:The most common dermatopathological feature was perivascular inflammation (37 cases, 88%), followed by epidermal atrophy (22 cases, 52%) and melanocyte loss (20 cases, 48%), basal vacuolization (15 cases, 36%). The incidence of basal vacuolization ( χ2=9.110, P=0.022), interface dermatitis ( χ2=11.672, P=0.005) and mucin deposition ( χ2=7.795, P=0.029) were significantly different in patients with myositis specific antibody (MSA) subgroup. The patients with positive tranional intermediary factor-1 (anti-TIF1-γ) antibody had higher incidence of interface dermatitis and basal vacuolization, and patients with melanoma differentiation-associated gene 5 (anti-MDA5) antibody had lower incidence of interface dermatitis. Interface dermatitis was positively associated with epidermal atrophy ( r=0.371, P=0.016) and parakeratosis ( r=0.316, P=0.041). Pigment inco-ntinence was positively associated with basal vacuolization ( r=0.384, P=0.012). Multi-factor line regression showed interface dermatitis was positively related to cutaneous disease area and severity index (CDASI). Conclusion:The dermatopathological features is different in subgroup of patients with DM ( β=10.295, P=0.004). Interface dermatitis is a marker of cutaneous disease activity, and its pathogenesis may be different from that of perivascular inflammation. Keratinocytes may be involved in the pathological process in interface dermatitis.

Objective:To determine the serum levels of chemokine CCL27 and its clinical relevancein patients with dermatomyositis (DM).Methods:The serum CCL27 levels of 58 DM patients, 21 polymyositis (PM) patients, 20 systemic lupus erythematosus (SLE) patients and 31 healthy controls were measured by enzyme linked immunosorbent assay (ELISA). The score of disease activity was measured by two physicians-based on the myositis disease activity assessment tool (MDAAT). Its correlation with serum levels of CCL27 was analyzed. The difference between multiple groups were compared using analysis of variance (ANOVA) and t test, and the relevance was analyzed using Spearman correlation analysis and generalized method of moments (GEE) model. Results:Theserum level of CCL27 in DM patients (178±49) pg/ml was significantly higher than PM (110±40) pg/ml, SLE (141±46) pg/ml and healthy controls (137±38) pg/ml ( F=14.192, P<0.01). Crosssectional analysis showed that the serum CCL27 levelwaspositively correlated with global disease activity ( r=0.301, P=0.022) andskin disease activity ( r=0.493, P<0.01). Patients with V sign had higher serum CCL27 levels (191±52) pg/ml than the patients without (153±33) pg/ml ( t=2.839, P<0.01). Patients with holster sign had higher serum CCL27 levels (196±58) pg/ml than the patients without (168±41) pg/ml ( t=2.176, P=0.034). Follow-up study also found that CCL27l evels were positively correlated with global disease activity ( β=0.031, P=0.042) and skin disease activity ( β=0.032, P<0.01). Conclusion:The serum CCL27 levels are increased in patients with DM and can reflect the skin disease activity. The results of this study suggest that CCL27 may be a marker for cutaneous damage and monitoring of therapeutic effect.

Objective:To investigate the pathological characteristics of skeletal muscle and its association with anti-aminoacyl-tRNA synthetases(ARS) antibodies and clinical features in patients with anti-synthetase syndrome (ASS).Methods:Patients diagnosed as ASS at China-Japan Friendship Hospital from 2008 to 2018 were involved in this study. Immunohistochemistry staining of MHC-Ⅰ, CD3, CD4, CD8 and CD20 molecule were performed in all muscle specimen taken from these patients. According to the muscular pathological characteristics, all patients could be divided into six pathological subgroups: pathologic DM(pDM), pathologic PM (pPM), unspecified myositis(USM), necrotizing myopathies (NAM), only MHC-Ⅰexpression (MHC-Ⅰ) and normal pathology groups. Immunoblotting assay was used to detect anti-ARS antibodies. Statistical analysis was performed using Statistical Product and Service Solutions (SPSS) software. T-test, Mann-Whitney U test and Analysis of Variance (ANOVA) were used for the comparison of measurement data. Chi-square test or Fisher's test were used for categorical data. P<0.05 was considered statistically significant. Results:A total of 77 patients underwent muscle biopsy and anti-ARS antibodies test, with the average age of (50±12) years and disease course of 9(3-24) months. The prevalence of anti-Jo-1, anti-PL-7, anti-PL-12, anti-EJ and anti-OJ antibodies in these patients was 47%(36 cases), 29%(22 cases), 12%(9 cases), 13%(10 cases) and 0 respectively. Among all the ASS patients, the most common pathological type was USM(37/77, 48%), followed by pDM(14/77, 18%), the normal pathology(13/77, 17%), NAM(10/77, 13%) and MHC-Ⅰ (3/77, 4%) groups. There were no pPM subtypes in all groups. The frequency of pDM types was significantly different among the anti-ARS antibodies groups( χ2=9.075, P=0.028). The anti-EJ-positive patients had a higher frequency of pDM compared with anti-PL-7-group(40% vs 4%, χ2=6.555, P=0.024). Meanwhile, the CD20 expression in muscle tissue was observed in 30% of anti-EJ-positive patients and 4% of anti-Jo-1-positive ones. There was statistically significant difference in the positive rate of CD20 expression among anti-ARS antibodies groups ( χ2=12.837, P<0.01). Conclusion:The muscle pathological characteristics of ASS are polymorphic and relate to the anti-ARS antibodies. Performing muscle biopsy and distinguishing pathological types are helpful for the diagnosis and stratification of ASS.

Objective To investigate the association of distinct myositis specific autoantibodies (MSAs) with long-term survival of patients with polymyositis (PM) and dermatomyositis (DM).Methods We analyzed the clinical data and outcome of patients with PM and DM who were hospita-lized in the department of rheumatology of China-Japan Friendship hospital from 1994 to 2015,and evaluated the impact of MSAs on the prognosis of patients.Multivariate Cox regression analysis was used to identify the prognostic risk factors for PM/DM patients.Results A total of 383 PM/DM patients were followed up for 1-333 months.Cumulative survival and 10-year survival rate of all patients were 68.6％ and 76.2％,respectively.The survival rate of 80.4％ and 77.1％ at 3 and 5 years in patients with MSAs,which were lower than those of patients with-out MSAs,who had the survival rate of 90.1％ and 87.4％ at 3 and 5 years,respectively(x2=3.90 and 3.98,P＜0.05).There was significant difference for long-term survival in all MSAs positive groups (x2=40.654,P=0.000).Anti-MDA5 positive patients who had the 10-year survival rate of 28.7％ had the worst prognosis,while anti-HMGCR positive patients who had the l0-year survival rate of 100％ had the best outcome in all groups.Multivariate Cox regression analysis showed that independent risk factors associated with the long-term survival of patients were age of onset,complicated with malignancies,dysphagia,rapidly progress interstitial lung disease,antiMDA5 antibody positive,increased serum aspartate transferase and C reaction protein.Conclusion MSAs are strongly associated with the prognosis of patients with PM/DM.Patients with MSAs has worse 5-year overall survival than those without MSAs,which indicates that screening MSAs and aggressive treatment for PM/DM patients at very early stage of disease may improve the outcome.

Objective To identify clinical features and risk factors in adult dermatomyositis (DM) with calcinosis.Methods Four hundred and eighty patients' clinical data were collected.The correlation between calcinosis and no calcinosis in adult DM were calculated by t test,x2 test and Mann-whitney U test.Multifactor logistic regression model was established to analyze independent factors for adult DM with calcinosis.Results Calcinosis occurred in 22 cases among these 480 patients with DM.The incidence of adult DM with calcinosis was 4.6％.Calcinosis was most common in the extremities and trunks.Patients with calcinosis had a longer disease duration [48 (24,120) months vs 10 (3,24) months,U=1993,P=0.000)] and more myothenia (95.5％ vs 76.9％,x2=4.192,P=0.038),panniculitis (9.1％ vs 0.4％,P=0.011),periungual erythematosus (22.7％ vs 5.89％,x2=7.044,P=0.008),skin ulcer (50.0％ vs 5.2％,x2=55.767,P=0.000),Raynaud's phenomenon (27.5％ vs 8.1％,x2=3.956,P=0.047).The anti-NXP2 antibody (27.3％ vs 3.9％,x2=19.416,P=0.000) and antiMDA5 antibody (31.8％ vs 12.9％,x2=4.851,P=0.028) were more frequently found in patients with calcinosis.Multi-factor logistic regression showed that anti-NXP2 antibody [OR=10.899,95％CI (2.593,45.816),P=0.001],long diseases duration [OR=1.105,95％CI(1.008,1.021),P=0.000] and skin ulcer [OR=31.585,95％CI(10.683),93.387,P=0.000] were risk factors for adult DM with calcinosis.Conclusion The incidence of calcinosisis in adult DM is 4.5％ in our cohort.Patients with calcinosis are adistinct clinical subset of adult DM.Long disease duration,skin ulcer and anti-NXP2 positive are independent risk factors for adult DM with calcinosis.

Objective To investigate the relationship between BRCA1 protein expression in lung cancer tissues and the effect of gemcitabine and Cisplatin based chemotherapy.Methods 80 cases of squamous cell carcinoma confirmed by pathology from May 2013 to May 2015 were selected, and treated with gemcitabine plus cisplatin chemotherapy and Ginseng and Astragalus assisted theropy.BRCA1 protein expression of all patients were detected, and relationship between the effect of chemotherapy and prognosis of patients with BRCA1 protein expression in lung cancer tissues were studied.Results In 80 cases, BRCA1 protein was positive in 40 cases, 40 cases were negative, CR 5 patients, PR 6 patients with BRCA1 positive expression, the remission rate was lower than the BRCA1 negative patients ( P <0.05 ) .80 cases of adverse reactions were seen as leukopenia, gastrointestinal symptoms (nausea and vomiting), abnormal liver function, BRCA1 expression of peripheral neurotoxicity, the incidence of complications in patients with positive expression were significantly higher than BRCA1 negative patients(P<0.05).N stage (P=0.03), BRCA1 gene (P=0.02) were independent risk factors of the prognosis of patients.Conclusion BRCA1 protein positive patients in lung cancer tissue had poor chemotherapy effect, more adverse reactions, and poor prognosis.

Objective The aim of this study is to analyze the prevalence of myositis-specific autoantibodies (MSAs) and to elucidate their associations with clinical features in Chinese dermatomyositis (DM) patients. Methods Four hundreds and twenty-seven DM patients were enrolled in this retrospective study. Clinical features and sera were collected. Twelve subtypes of MSAs were detected by commercial test kits. The correlations between MSAs and clinical phenotypes in DM patients were calculated by t test, Mann-Whitney U test or χ2 test. In order to clarify whether MSAs subsets would be independent factors of certain clinical feature or not, separate models were established to test the correlation via the Logistic regression analysis. Results The positivity of MSAs was 69.8% in 427 patients with DM. Anti-ARS, anti-MDA5 and anti-TIF1-γ antibodies were the three most common MSAs in the DM patients with positivity of 19.9%, 17.6%and 17.1% respectively. Different kinds of rash associated with MSAs subtypes by χ2 test. Certain MSAs subtype might be an independent factor for clinical features via logistic regression analysis. Interstitial lung disease (ILD) was observed more frequently in patients carrying anti-MDA5 [OR=5.266, 95%CI (2.522, 10.996), P<0.01] and anti-Jo-1 [OR=6.232, 95%CI (1.674, 23.199), P=0.006]. On the contrary, anti-Mi2 [OR=0.208, 95%CI (0.074, 0.580, P=0.003] and anti-TIF1-γ [OR=0.189, 95%CI (0.096, 0.370), P<0.01] were protective factors against developing ILD. Anti-TIF1-γ was an independent risk factor for cancer-associated myositis [OR=5.907, 95%CI (2.868, 12.168), P<0.01]. Anti-TIF1-γ[OR=2.789, 95%CI (1.594, 4.880) P<0.01], anti-NXP2 [OR=2.983, 95%CI (1.274, 6.982), P=0.012] and anti-SAE1 [OR=4.815, 95%CI (1.082, 21.424), P=0.039] could worsen dysphagic tendencies. In contrast, anti-MDA5 [OR=0.349, 95%CI (0.169, 0.720), P=0.004] might decrease the prevalence of this manifestation. Conclusion Patients with DM have a high frequency of MSAs. Some subtypes of MSAs are correlated with and may be independent factors of different clinical phenotypes. These indicated that MSAs can be useful biomarkers in monitoring the extramuscular features in DM patients.

Objective To analyze the clinical effect of combination therapy of somatidine on colorectal cancer with intestinal obstruction. Methods 120 patients with colorectal cancer complicated with intestinal obstruction who were treated with Yujiang County Hospital of Zhejiang Province from November 2016 to May 2017 were selected as the subjects of this study and divided into observation group by dynamic random color ball extraction method And control group. The rats in the control group were given stage I colonic resection and anastomosis. The observation group was treated with somatanine combined with stage I colonic resection and anastomosis. The differences of the indexes and complication were compared between the two groups. Results There was no significant difference in the operation time between the two groups (3.01 ± 0.41) days and the hospitalization time (10.11 ± 1.21) days in the observation group, and the time of operation was significantly higher than that of the control group (5.41 ± 0.84) days, the hospitalization time was (16.33 ± 2.05) days, the number of complications occurred in 10 cases, the difference was more than that in the control group Significantly(P<0.05). Conclusion Si Tanning combined with surgical treatment of colorectal cancer with intestinal obstruction has a certain value.

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.