AIM:Primary biliary cirrhosis (PBC) is a chronic, cholestatic disease of autoimmune etiology,the histology of which shows a destruction of the intrahepatic bile duct and portal inflammation. Ursodeoxycholic acid (UDCA...AIM:Primary biliary cirrhosis (PBC) is a chronic, cholestatic disease of autoimmune etiology,the histology of which shows a destruction of the intrahepatic bile duct and portal inflammation. Ursodeoxycholic acid (UDCA) is now used as a first-line drug for asymptomatic PBC (aPBC) because it is reported that UDCA decreases mortality and prolongs the time of liver transplantation.However, only 20-30% of patients respond fully to UDCA.Recently,lipoprotein-lowering agents have been found to be effective for PBC.The aim of this study was to examine the safety and efficacy of fenofibrate, a member of the fibrate class of hypolipidemic and anti-inflammatory agent via peroxysome proliferatory-activated receptor α,in patients with aPBC.METHODS:Fenofibrate was administered for twelve weeks in nine patients with aPBC who failed to respond to UDCA.UDCA was used along with fenofibrate during the study.The data from aPBC patients were analyzed to assess the biochemical effect of fenofibrate during the study.RESULTS: The serum levels of alkaline phosphatase (ALP)(285±114.8IU/L) and immunoglobulin M (IgM) (255.8±85.9mg/dl) significantly decreased to 186.9±76.2IU/L and 192.9±67.5mg/dL respectively, after fenofibrate treatment in patients with aPBC (P<0.05). Moreover,the titer of antimitochondrial antibody (AMA) also decreased in 4 of 9 patients with aPBC. No adverse reactions were observed in any patients.CONCLUSION:Fenofibrate appears to be significantly effective in treating patients with aPBC who respond incompletely to UDCA alone.Although the mechanism of fenofibrate on aPBC has not yet been fully clarified,combination therapy using fenofibrate and UDCA might be related to the anti-immunological effects, such as the suppression of AMA production as well as its antiinflammatory effect.展开更多
AIM： To investigate the possible relationships between gastric autoimmune phenomena and clinical presentations of this disorder, in consecutive atrophic body gastritis patients. METHODS： A total of 140 atrophic body...AIM： To investigate the possible relationships between gastric autoimmune phenomena and clinical presentations of this disorder, in consecutive atrophic body gastritis patients. METHODS： A total of 140 atrophic body gastritis patients, diagnosed as consecutive outpatients presenting with macrocytic or iron deficiency anemia, or longstanding dyspepsia underwent gastroscopy with antral and body biopsies, assay of intrinsic factor, parietal cells and Helicobacter pylori （ H pylon） antibodies. Gastritis was assessed according to Sydney System. RESULTS： Parietal cell antibodies were equally distributed in all clinical presentations, whereas the positivity of intrinsic factor antibodies （49/140, 35%） was significantly higher in pernicious anemia patients （49.2%） than in iron deficiency （21.1%） and dyspeptic patients （27.8%）. No specific pattern of autoantibodies was related to the clinical presentations of atrophic body gastritis. A positive correlation was obtained between the body atrophy score and the intrinsic factor antibody levels （r=0.2216, P=0.0085）. Associated autoimmune diseases were present in 25/140 （17.9%） patients, but the prevalence of autoimmune diseases was comparable, irrespective of the clinical presentations. CONCLUSION： The so-called hallmarks of gastric autoimmunity, particularly in intrinsic factor antibody cannot be usefully employed in defining an autoimmune pattern in the clinical presentations of ABG.展开更多
Liver diseases in pregnancy may be categorized into liver disorders that occur only in the setting of pregnancy and liver diseases that occur coincidentally with pregnancy. Hyperemesis gravidarum, preeclampsia/eclamps...Liver diseases in pregnancy may be categorized into liver disorders that occur only in the setting of pregnancy and liver diseases that occur coincidentally with pregnancy. Hyperemesis gravidarum, preeclampsia/eclampsia, syndrome of hemolysis, elevated liver tests and low platelets (HELLP), acute fatty liver of pregnancy, and intrahepatic cholestasis of pregnancy are pregnancy-specific disorders that may cause elevations in liver tests and hepatic dysfunction. Chronic liver diseases, including cholestatic liver disease, autoimmune hepatitis, Wilson disease, and viral hepatitis may also be seen in pregnancy. Management of liver disease in pregnancy requires collaboration between obstetricians and gastroenterologists/hepatologists. Treatment of pregnancy-specific liver disorders usually involves delivery of the fetus and supportive care, whereas management of chronic liver disease in pregnancy is directed toward optimizing control of the liver disorder. Cirrhosis in the setting of pregnancy is less commonly observed but offers unique challenges for patients and practitioners. This article reviews the epidemiology, pathophysiology, diagnosis, and management of liver diseases seen in pregnancy.展开更多
SJOGREN＇S syndrome （SS） is a chronic inflammatory autoimmune disease characterized by the infiltration of lymphocytes and plasma cells in exocrine glands, especially salivary and lacrimal gland interstitium. The cl...SJOGREN＇S syndrome （SS） is a chronic inflammatory autoimmune disease characterized by the infiltration of lymphocytes and plasma cells in exocrine glands, especially salivary and lacrimal gland interstitium. The clinical manifestations of SS are complex. When the digestive system is involved,展开更多
Objective.To investigate the pathological significance of tubuloreticular structure(TRS) and cylindri- cal confronting cisternae(CCC) in patients with lupus nephritis. Methods. An electron microscopical study of 24 re...Objective.To investigate the pathological significance of tubuloreticular structure(TRS) and cylindri- cal confronting cisternae(CCC) in patients with lupus nephritis. Methods. An electron microscopical study of 24 renal biopsy specimens from patients with lupus nephritis was carried out, with particular emphasis on two endoplasmic reticulum(ER)-related structures. Result. TRS was found in 18 cases, and CCC in 10 of them. TRS often appeared in the capillary en- dothelium,and did not correlate well with the activity index of lupus nephritis. CCC appeared frequently in monocyte/macrophage and lymphocyte, and correlated well with both the activity index and the amount of interstitial immune deposits. Conclusion.TRS and CCC derived from inward "budding" of ER membrane were suggested and the morphogenesis and morphologic variations of CCC were discussed. Both TRS and CCC are pathognomonic, though not specific changes. They may be helpful in pathologic diagnosis of lupus nephritis, when properly combined with certain clinical and pathological features.展开更多
Celiac disease (CD) is an autoimmune disorder of the small intestine that occurs in genetically predisposed people at all ages.However,it can be associated also to other immunopathological disorders,and may be associa...Celiac disease (CD) is an autoimmune disorder of the small intestine that occurs in genetically predisposed people at all ages.However,it can be associated also to other immunopathological disorders,and may be associated with abnormal histology in segments of the gut other than the small bowel including colonic inflammation.While guidelines for endoscopic investigation of the jejunum are well defined,no indication is defined for colonic investigation.We describe four cases of concurrent CD and microscopic colitis (MC) diagnosed at our department over a 10-year period and analyzed the main features and outcomes of CD in this setting.The symptoms of these patients were improved initially by a gluten-free diet before the onset of MC symptoms.Two of the patients were siblings and had an atypical form of CD.The other two patients with CD and MC also presented with fibrosing alveolitis and were anti-Saccharomyces cerevisiae antibody positive.The co-existence of immune-mediated small bowel and colonic inflammatory and pulmonary diseases are not well-known,and no systematic approach has been used to identify the lifelong patterns of these immune-based diseases.Patients can develop,or present with CD at any stage in life,which can co-exist with other gastrointestinal diseases of (auto-) immune origin.In addition,the fa-milial co-existence and prevalence of MC in patients with a prior diagnosis of CD are unclear.Clinicians managing celiac disease should be aware of these associations and understand when to consider colon investigation.展开更多
文摘AIM:Primary biliary cirrhosis (PBC) is a chronic, cholestatic disease of autoimmune etiology,the histology of which shows a destruction of the intrahepatic bile duct and portal inflammation. Ursodeoxycholic acid (UDCA) is now used as a first-line drug for asymptomatic PBC (aPBC) because it is reported that UDCA decreases mortality and prolongs the time of liver transplantation.However, only 20-30% of patients respond fully to UDCA.Recently,lipoprotein-lowering agents have been found to be effective for PBC.The aim of this study was to examine the safety and efficacy of fenofibrate, a member of the fibrate class of hypolipidemic and anti-inflammatory agent via peroxysome proliferatory-activated receptor α,in patients with aPBC.METHODS:Fenofibrate was administered for twelve weeks in nine patients with aPBC who failed to respond to UDCA.UDCA was used along with fenofibrate during the study.The data from aPBC patients were analyzed to assess the biochemical effect of fenofibrate during the study.RESULTS: The serum levels of alkaline phosphatase (ALP)(285±114.8IU/L) and immunoglobulin M (IgM) (255.8±85.9mg/dl) significantly decreased to 186.9±76.2IU/L and 192.9±67.5mg/dL respectively, after fenofibrate treatment in patients with aPBC (P<0.05). Moreover,the titer of antimitochondrial antibody (AMA) also decreased in 4 of 9 patients with aPBC. No adverse reactions were observed in any patients.CONCLUSION:Fenofibrate appears to be significantly effective in treating patients with aPBC who respond incompletely to UDCA alone.Although the mechanism of fenofibrate on aPBC has not yet been fully clarified,combination therapy using fenofibrate and UDCA might be related to the anti-immunological effects, such as the suppression of AMA production as well as its antiinflammatory effect.
基金Supported by Grants From the Italian Ministry for the University (MIUR), No. 02/12/01/10 1999-2002 and No. 8.111.126.5
文摘AIM： To investigate the possible relationships between gastric autoimmune phenomena and clinical presentations of this disorder, in consecutive atrophic body gastritis patients. METHODS： A total of 140 atrophic body gastritis patients, diagnosed as consecutive outpatients presenting with macrocytic or iron deficiency anemia, or longstanding dyspepsia underwent gastroscopy with antral and body biopsies, assay of intrinsic factor, parietal cells and Helicobacter pylori （ H pylon） antibodies. Gastritis was assessed according to Sydney System. RESULTS： Parietal cell antibodies were equally distributed in all clinical presentations, whereas the positivity of intrinsic factor antibodies （49/140, 35%） was significantly higher in pernicious anemia patients （49.2%） than in iron deficiency （21.1%） and dyspeptic patients （27.8%）. No specific pattern of autoantibodies was related to the clinical presentations of atrophic body gastritis. A positive correlation was obtained between the body atrophy score and the intrinsic factor antibody levels （r=0.2216, P=0.0085）. Associated autoimmune diseases were present in 25/140 （17.9%） patients, but the prevalence of autoimmune diseases was comparable, irrespective of the clinical presentations. CONCLUSION： The so-called hallmarks of gastric autoimmunity, particularly in intrinsic factor antibody cannot be usefully employed in defining an autoimmune pattern in the clinical presentations of ABG.
文摘Liver diseases in pregnancy may be categorized into liver disorders that occur only in the setting of pregnancy and liver diseases that occur coincidentally with pregnancy. Hyperemesis gravidarum, preeclampsia/eclampsia, syndrome of hemolysis, elevated liver tests and low platelets (HELLP), acute fatty liver of pregnancy, and intrahepatic cholestasis of pregnancy are pregnancy-specific disorders that may cause elevations in liver tests and hepatic dysfunction. Chronic liver diseases, including cholestatic liver disease, autoimmune hepatitis, Wilson disease, and viral hepatitis may also be seen in pregnancy. Management of liver disease in pregnancy requires collaboration between obstetricians and gastroenterologists/hepatologists. Treatment of pregnancy-specific liver disorders usually involves delivery of the fetus and supportive care, whereas management of chronic liver disease in pregnancy is directed toward optimizing control of the liver disorder. Cirrhosis in the setting of pregnancy is less commonly observed but offers unique challenges for patients and practitioners. This article reviews the epidemiology, pathophysiology, diagnosis, and management of liver diseases seen in pregnancy.
文摘SJOGREN＇S syndrome （SS） is a chronic inflammatory autoimmune disease characterized by the infiltration of lymphocytes and plasma cells in exocrine glands, especially salivary and lacrimal gland interstitium. The clinical manifestations of SS are complex. When the digestive system is involved,
文摘Objective.To investigate the pathological significance of tubuloreticular structure(TRS) and cylindri- cal confronting cisternae(CCC) in patients with lupus nephritis. Methods. An electron microscopical study of 24 renal biopsy specimens from patients with lupus nephritis was carried out, with particular emphasis on two endoplasmic reticulum(ER)-related structures. Result. TRS was found in 18 cases, and CCC in 10 of them. TRS often appeared in the capillary en- dothelium,and did not correlate well with the activity index of lupus nephritis. CCC appeared frequently in monocyte/macrophage and lymphocyte, and correlated well with both the activity index and the amount of interstitial immune deposits. Conclusion.TRS and CCC derived from inward "budding" of ER membrane were suggested and the morphogenesis and morphologic variations of CCC were discussed. Both TRS and CCC are pathognomonic, though not specific changes. They may be helpful in pathologic diagnosis of lupus nephritis, when properly combined with certain clinical and pathological features.
文摘Celiac disease (CD) is an autoimmune disorder of the small intestine that occurs in genetically predisposed people at all ages.However,it can be associated also to other immunopathological disorders,and may be associated with abnormal histology in segments of the gut other than the small bowel including colonic inflammation.While guidelines for endoscopic investigation of the jejunum are well defined,no indication is defined for colonic investigation.We describe four cases of concurrent CD and microscopic colitis (MC) diagnosed at our department over a 10-year period and analyzed the main features and outcomes of CD in this setting.The symptoms of these patients were improved initially by a gluten-free diet before the onset of MC symptoms.Two of the patients were siblings and had an atypical form of CD.The other two patients with CD and MC also presented with fibrosing alveolitis and were anti-Saccharomyces cerevisiae antibody positive.The co-existence of immune-mediated small bowel and colonic inflammatory and pulmonary diseases are not well-known,and no systematic approach has been used to identify the lifelong patterns of these immune-based diseases.Patients can develop,or present with CD at any stage in life,which can co-exist with other gastrointestinal diseases of (auto-) immune origin.In addition,the fa-milial co-existence and prevalence of MC in patients with a prior diagnosis of CD are unclear.Clinicians managing celiac disease should be aware of these associations and understand when to consider colon investigation.
