Nodular regenerative hyperplasia(NRH)is a rare liver condition characterized by a widespread benign transformation of the hepatic parenchyma into small regenerative nodules.NRH may lead to the development of non-cirrh...Nodular regenerative hyperplasia(NRH)is a rare liver condition characterized by a widespread benign transformation of the hepatic parenchyma into small regenerative nodules.NRH may lead to the development of non-cirrhotic portal hypertension.There are no published systematic population studies on NRH and our current knowledge is limited to case reports and case series.NRH may develop via autoimmune,hematological,infectious,neoplastic,or drug-related causes.The disease is usually asymptomatic,slowly or nonprogressive unless complications of portal hypertension develop.Accurate diagnosis is made by histopathology,which demonstrates diffuse micronodular transformation without fibrous septa.Lack of perinuclear collagen tissue distinguishes NRH from typical regenerative nodules in the cirrhotic liver.While the initial treatment is to address the underlying disease,ultimately the therapy is directed to the management of portal hypertension.The prognosis of NRH depends on both the severity of the underlying illness and the prevention of secondary complications of portal hypertension.In this review we detail the epidemiology,pathogenesis,diagnosis,management,and prognosis of NRH.展开更多
Hepatitis C virus （HCV） has been recognized to be both a hepato- and lymphotropic virus. HCV lymphotropism represents an essential lap in the pathogenesis of virus-related autoimmune and lymphoproliferative disorder...Hepatitis C virus （HCV） has been recognized to be both a hepato- and lymphotropic virus. HCV lymphotropism represents an essential lap in the pathogenesis of virus-related autoimmune and lymphoproliferative disorders, ranging from clonal expansion of B-cells with organ-and non-organ-specific autoantibody production up to overt non-Hodgkin＇s lymphoma along a continuous step-by-step model of B-cell lymphomagenesis, where the intermediated mixed cryoglobulinemia could be considered as a stage of suppressible antigen-driven lymphoproliferation. HCV infection of lymphoid ceils could set up privileged reservoirs able to interfere with the host viral clearance efficiency and may be implicated in viral recurrence after apparently successful antiviral therapy. The HCV long-lasting extrahepatic replicative state generates an abnormal systemic immunological response, easily detectable by searching simple laboratory and clinical parameters, mainly represented by vasculitis-like skin features and hypocomplementemia.The presence or absence of this hypersensitivity pattern seems to correlate with the antiviral response and could be identified as a novel immunological cofactor. Further research is required to fully verify the real impact on therapeutic choice/regimen.展开更多
Triptolide is a natural, biologically active component derived from Chinese herb Tripterygium Wilfordii Hook F. (TWHF) which is effective in the clinical treatment of autoimmune diseases, however, the mechanisms by wh...Triptolide is a natural, biologically active component derived from Chinese herb Tripterygium Wilfordii Hook F. (TWHF) which is effective in the clinical treatment of autoimmune diseases, however, the mechanisms by which triptolide exerts immunosuppression remain fully understood. The primary of this study is to demonstrate whether triptolide can affect phenotype, cytokine production and allogeneic T cell-stimulatory capacity of dendritic cells (DCs) which are critical in the induction of immune response or tolerance. Phenotypic analysis show that triptolide does not affect the expression of MHC (Ia b), CD80, CD86 and CD40 of DC stimulated with or not LPS, but significantly inhibits IL-12p70 production by DC in a dose-dependent manner. Triptolide-treated DCs exhibit a reduced capacity to stimulate proliferation of allogeneic CD4 + T lymphocytes. Therefore, triptolide-mediated immunosuppression may due, in part, to the inhibition of IL-12p70 production and impairment of allogeneic T cell-stimulatory capacity of DCs. Our results may provide a possible mechanistic explanation for the effectiveness of triptolide in the treatment of autoimmune diseases.展开更多
Vitamin D is a seco-steroid involved in calcium and phosphorus metabolism,and bone formation and mineralization, through binding to a specific nuclear receptor,vitamin D receptor（VDR）.Besides its well-established fu...Vitamin D is a seco-steroid involved in calcium and phosphorus metabolism,and bone formation and mineralization, through binding to a specific nuclear receptor,vitamin D receptor（VDR）.Besides its well-established functions on bone health,multiple lines of evidence have indicated the immunomodulatory roles of vitamin D.Vitamin D can affect both innate and adaptive immunity,and prevent autoimmune responses efficiently.Vitamin D regulates the immune responses by suppressing T cell proliferation and modulating macrophage functions.Epidemiological studies have shown that vitamin D deficiency is associated with multiple diseases such as rickets and cancer.Moreover,associations between vitamin D and autoimmune diseases have been confirmed in multiple sclerosis（MS）,rheumatoid arthritis（RA）,etc.The present review mainly summarized the recent findings on the immunomodulatory role of vitamin D in various disorders,with special focus on its role in MS,an autoimmune disease of the nervous system.展开更多
Systemic lupus erythematosus(SLE) is a debilitating autoimmune disease that can involve multi-organs. B cells play a central role in the immunopathogenesis via antibody-dependent and antibody-independent ways. Excessi...Systemic lupus erythematosus(SLE) is a debilitating autoimmune disease that can involve multi-organs. B cells play a central role in the immunopathogenesis via antibody-dependent and antibody-independent ways. Excessive autoantibodies production, hyperresponsiveness and prolonged survival of autoreactive B cells are characteristics of SLE. In this article, mechanisms of self-tolerance loss of B cells and promising B cell-targeting therapies in lupus are reviewed and discussed.展开更多
文摘Nodular regenerative hyperplasia(NRH)is a rare liver condition characterized by a widespread benign transformation of the hepatic parenchyma into small regenerative nodules.NRH may lead to the development of non-cirrhotic portal hypertension.There are no published systematic population studies on NRH and our current knowledge is limited to case reports and case series.NRH may develop via autoimmune,hematological,infectious,neoplastic,or drug-related causes.The disease is usually asymptomatic,slowly or nonprogressive unless complications of portal hypertension develop.Accurate diagnosis is made by histopathology,which demonstrates diffuse micronodular transformation without fibrous septa.Lack of perinuclear collagen tissue distinguishes NRH from typical regenerative nodules in the cirrhotic liver.While the initial treatment is to address the underlying disease,ultimately the therapy is directed to the management of portal hypertension.The prognosis of NRH depends on both the severity of the underlying illness and the prevention of secondary complications of portal hypertension.In this review we detail the epidemiology,pathogenesis,diagnosis,management,and prognosis of NRH.
文摘Hepatitis C virus （HCV） has been recognized to be both a hepato- and lymphotropic virus. HCV lymphotropism represents an essential lap in the pathogenesis of virus-related autoimmune and lymphoproliferative disorders, ranging from clonal expansion of B-cells with organ-and non-organ-specific autoantibody production up to overt non-Hodgkin＇s lymphoma along a continuous step-by-step model of B-cell lymphomagenesis, where the intermediated mixed cryoglobulinemia could be considered as a stage of suppressible antigen-driven lymphoproliferation. HCV infection of lymphoid ceils could set up privileged reservoirs able to interfere with the host viral clearance efficiency and may be implicated in viral recurrence after apparently successful antiviral therapy. The HCV long-lasting extrahepatic replicative state generates an abnormal systemic immunological response, easily detectable by searching simple laboratory and clinical parameters, mainly represented by vasculitis-like skin features and hypocomplementemia.The presence or absence of this hypersensitivity pattern seems to correlate with the antiviral response and could be identified as a novel immunological cofactor. Further research is required to fully verify the real impact on therapeutic choice/regimen.
文摘Triptolide is a natural, biologically active component derived from Chinese herb Tripterygium Wilfordii Hook F. (TWHF) which is effective in the clinical treatment of autoimmune diseases, however, the mechanisms by which triptolide exerts immunosuppression remain fully understood. The primary of this study is to demonstrate whether triptolide can affect phenotype, cytokine production and allogeneic T cell-stimulatory capacity of dendritic cells (DCs) which are critical in the induction of immune response or tolerance. Phenotypic analysis show that triptolide does not affect the expression of MHC (Ia b), CD80, CD86 and CD40 of DC stimulated with or not LPS, but significantly inhibits IL-12p70 production by DC in a dose-dependent manner. Triptolide-treated DCs exhibit a reduced capacity to stimulate proliferation of allogeneic CD4 + T lymphocytes. Therefore, triptolide-mediated immunosuppression may due, in part, to the inhibition of IL-12p70 production and impairment of allogeneic T cell-stimulatory capacity of DCs. Our results may provide a possible mechanistic explanation for the effectiveness of triptolide in the treatment of autoimmune diseases.
基金supported by thegrant from China Scholarship Council(Grant No.[2008]3019)
文摘Vitamin D is a seco-steroid involved in calcium and phosphorus metabolism,and bone formation and mineralization, through binding to a specific nuclear receptor,vitamin D receptor（VDR）.Besides its well-established functions on bone health,multiple lines of evidence have indicated the immunomodulatory roles of vitamin D.Vitamin D can affect both innate and adaptive immunity,and prevent autoimmune responses efficiently.Vitamin D regulates the immune responses by suppressing T cell proliferation and modulating macrophage functions.Epidemiological studies have shown that vitamin D deficiency is associated with multiple diseases such as rickets and cancer.Moreover,associations between vitamin D and autoimmune diseases have been confirmed in multiple sclerosis（MS）,rheumatoid arthritis（RA）,etc.The present review mainly summarized the recent findings on the immunomodulatory role of vitamin D in various disorders,with special focus on its role in MS,an autoimmune disease of the nervous system.
文摘Systemic lupus erythematosus(SLE) is a debilitating autoimmune disease that can involve multi-organs. B cells play a central role in the immunopathogenesis via antibody-dependent and antibody-independent ways. Excessive autoantibodies production, hyperresponsiveness and prolonged survival of autoreactive B cells are characteristics of SLE. In this article, mechanisms of self-tolerance loss of B cells and promising B cell-targeting therapies in lupus are reviewed and discussed.
