Recommendations for management of antiphospholipid syndrome in China / 中华内科杂志

Antiphospholipid syndrome (APS) is a systemic autoimmune disorder with vascular, obstetric, and hematological manifestations associated with thrombotic and inflammatory mechanisms orchestrated by antiphospholipid (aPLs) antibodies. Current clinical practice in APS is highly variable duo to lack of high quality of evidence. Here, Chinese Rheumatology Association developed recommendations for management of APS in China. The recommendations cover the early diagnosis, disease evaluation, thrombotic risk assessment, and treatment.

Expression and significance of non-canonical pyroptosis pathway-related proteins in muscle tissues of patients with dermatomyositis/polymyositis / 中华微生物学和免疫学杂志

Objective:To detect the expression of cysteinyl aspartate specific proteinase 4 (caspase-4), caspase-5, gasdermin D (GSDMD), nucleotide-binding oligomerization domain-like receptor protein-3 (NLRP3), Pannexin-1 and P2X7 involved in non-canonical pyroptosis pathway in muscle tissues of patients with dermatomyositis (DM)/polymyositis (PM) and to investigate the roles and significance of them in the pathogenesis of DM and PM.Methods:Altogether 13 DM patients, nine PM patients and 20 volunteers (control group) treated in the Affiliated Hospital of Qinghai University from January 2019 to September 2020 were enrolled in the present study. The 20 volunteers with no additional concomitant diseases underwent debridement due to simple orthopedic trauma. Pathological changes in muscle tissues were detected by hematoxylin & eosin (HE) staining. Expression of caspase-4, caspase-5, GSDMD, NLRP3, Pannexin-1 and P2X7 in muscle tissues was measured using immunohistochemistry (IHC).Results:(1) HE staining results showed that the muscle fibers in the control group had basically normal morphology and structure with no obvious inflammatory cell infiltration, atrophy, degeneration or necrosis. However, the size and thickness of muscle fibers in DM and PM groups were different with excessive inflammatory cell infiltration, atrophy, degeneration and necrosis to varying degrees. Moreover, the pathological scores of HE staining in muscle tissues of DM and PM groups were significantly higher than that of the control group and the differences were of statistical significance ( P<0.05). (2) IHC staining results suggested that the expression of caspase-4, caspase-5, GSDMD, NLRP3, Pannexin-1 and P2X7 in muscle tissues was higher in DM and PM groups than in the control group ( P<0.05). (3) As indicated by Pearson correlation analysis, the pathological scores of HE staining in muscle tissues of DM and PM groups were positively correlated with the IHC scores of caspase-4, caspase-5, GSDMD, NLRP3, PAnnexin-1 and P2X7 ( P<0.05). Furthermore, the IHC scores of caspase-4 and caspase-5 were positively correlated with the IHC scores of GSDMD and Pannexin-1 ( P<0.05); the IHC score of GSDMD was positively correlated with the IHC score of NLRP3 ( P<0.05); the IHC score of Pannexin-1 was positively correlated with the IHC score of P2X7 in muscle tissues ( P<0.05). Conclusions:The non-canonical pyroptosis pathway might be involved in the pathogenesis of DM and PM, which was possibly achieved by promoting inflammatory response. These results suggested that the non-canonical pyroptosis pathway played crucial roles in the immune pathogenesis of DM and PM.

The expression and significance of cysteine-rich 61, connective tissue growth factor, vascular endothelial growth factor and microvascular density marked by CD105 in muscle tissue of patients with dermatomyosits and polymyosits / 中华风湿病学杂志

Objective By detecting vascular cysteine-rich 61(Cyr61) related factor,connective tissue growth factor (CTGF),vascular endothelial growth factor (VEGF) and CD105 markers of microvascular density (MVD) of muscle tissue in patients with PM/DM,the role and significance of the expression of Cyr61,CTGF,VEGF and CD105 in the process of vascular lesions of dermatomyosits (DM) and polymyosits (PM) were discussed.Methods The expression of Cyr61,CTGF,VEGF and CD105 markers of micro vascular density (MVD) were detected in 10 cases of DM,10 cases of PM and 20 controls by using immunohistochemical Envision two step method.Data were analyzed using Statistical Product and Service Solutions (SPSS) statistical software.Fisher's exact probability analysis and Spearman correlation analysis were conducted.Results Compared with the control group,Cyr61,CTGF,VEGF positive expression rate in muscle tissue of patients with DM and PM group were significantly different (P＜0.01),the positive expression rates of Cyr61,CTGF,VEGF in DM group and PM group were 90％,70％,90％,80％,80％,70％,and the control group (5％,10％,5％) respectively.In the muscle tissue of patients with DM and PM group,CD105 markers of capillaries could be seen,and MVD in DM and PM group were higher than that in the control group,the difference was statistically significant (F=8.103,P=0.001).Cyr61,CTGF and VEGF protein expression levels in muscle tissueof patients with DM and PM were positively correlated with MVD.Conclusion The muscle tissue of PM/DMpatients may have new blood vessels formation.Cyr61,CTGF,VEGF may be involved in the formation of newblood vessels in the PM/DM muscle tissue.The results of this study suggest that microvascular lesion plays animportant role in the immune pathogenesis of inflammatory myopathy such as PM/DM.

Determination and significance of monocyte chemotactic protein-3 and microvessel density in muscular tissue of patients with dermatomyosits and polymyosits / 实用医学杂志

Objective To determine the levels of monocyte chemotactic protein-3(MCP-3) and microvessel density (MVD) labeled by CD34 in skeletal muscle of patients with dermatomyositis (DM) and polymyositis (PM),so as to investigate the pathogenesis of DM/PM.Methods The expression of MCP-3 and MVD were detected in DM/PM patients and the healthy controls by using the immunohistochemical method.Results The positive expression rate of MCP-3 in DM group and PM group were higher than that in the control group (P ＜ 0.001,respectively).The microvessel marked by CD34 could be observed in muscle tissue of patients in DM group and PM group,and MVD in DM group and PM group was higher than that in the control group (P ＜ 0.001).No significant differences in the level of MCP-3 and MVD in the muscle tissue were found between the DM group and the PM group.The positive correlation between MCP-3 protein expression and MVD changes in the muscle tissue of patients inDM group and PM group was revealed (r =0.685/0.634,P ＜ 0.05).Conclusion The muscle tissue of DM/PM patients may have a new blood vessel formation,MCP-3 may be involved the process of new blood vessel formation in DM/PM.

IL-18 IFN-γ expression in labial glands and blood of patients with primary Sj(o)ren's syndrome / 中华风湿病学杂志

ObjectiveThis study explored the change of IL-18,and IFN-γ and their role in the pathogenesis of primary Sj(o)gren's syndrome (pSS) by determining the expression of IL-18,IFN-γ in labial glands and blood of patients with pSS.MethodsThe levels of IL-18,IFN-γ in labial glands of patients with pSS were determined by immunohistochemical method.The level of serum IL-18 was determined with enzyme linked immunosorbent assay ELISA.The level of γ globulin was measured by the cellulose acetate membrane method.SPSS 17.0 statistical software was used for data analysis.The t test,Fisher's exact probability test and index correlation analysis were performed with linear correlation analysis.Spearman's test was used to examine the association between the expression of IL-18 and IFN.ResultsThe positive rate of IL-18 and IFN-γ in patients with pSS was much higher than normal control group(P＜0.05).And the IL-18 and IFN-γ levels were positively correlated withpatients with pSS (P＜0.05).The level of serum IL-18 in patients with pSS was (216±85) pg/ml,which was much higher than normal control group(39±8) pg/ml.The IL-18 level and γ globulin(23.2±2.6)％ was positively correlated in patients with pSS (r=0.538,P＜0.05).ConclusionThe expression of IL-18 and IFN-γ in labial glands of patients with pSS are much higher in patients with pSS than those of the normal controls.Theresults have shown that IL-18 and IFN-γ may be involved inthe pathogenesis of pSS.