1/34. A case of autoimmune hypophysitis associated with asymptomatic primary biliary cirrhosis.We report a 61-year old male patient with panhypopituitarism complicated with asymptomatic primary biliary cirrhosis (PBC). T1-weighted magnetic resonance imaging demonstrated high intensity of the anterior pituitary gland. There was no mass lesion or enlargement of the pituitary gland or the stalk. Immunoblot analysis of the patient's sera with rat pituitary antigens revealed a band with a molecular size of 22 kD. Anti-M2 mitochondrial antibody has been consistently positive for five years. Liver biopsy revealed portal hepatitis with periportal infiltration of the inflammatory cells. This is the first case report of autoimmune hypophysitis complicated with asymptomatic PBC.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
2/34. Immunological studies in familial primary biliary cirrhosis.Tests for cell-mediated immunity and presence of autoantibodies were performed in a mother and daughter with primary biliary cirrhosis. lymphocytes transformation to phytohemagglutinin, delayed cutaneous response to one or more skin test antigens, and percentage of peripheral T and B lymphocytes were normal in both patients. Although successful in the mother, dinitrochlorbenzene sensitization was not achieved in the daughter. histocompatibility antigens 1 and 8, elevated levels of IgM, and antibodies to mitochondria, smooth muscle, and skeletal muscle were present in both patients. However, the clinical course was more severe in the daughter who developed portal hypertension with bleeding esophageal varices requiring portacaval anastomosis. Except for intermittent pruritus, the mother has remained asymptomatic.- - - - - - - - - - ranking = 2keywords = antigen (Clic here for more details about this article) |
3/34. Successful treatment with adefovir dipivoxil in a patient with fibrosing cholestatic hepatitis and lamivudine resistant hepatitis b virus.Fibrosing cholestatic hepatitis (FCH) is a severe clinical and histological variant of hepatitis b virus (HBV) infection seen most commonly in the HBV infected allograft after liver transplantation. Without treatment, FCH is fatal, rapidly and universally. Remission has been reported with lamivudine but is associated with evolving resistance to lamivudine. Adefovir dipivoxil has recently been reported to be a potent and highly effective inhibitor of HBV replication in both wild-type and lamivudine resistant HBV infection. We report a case of FCH 15 months after liver transplantation for HBV related cirrhosis despite therapy with lamivudine and hepatitis B immunoglobulin (HBIg). Within two weeks of commencing treatment with adefovir dipivoxil 10 mg once daily, the patient had made a remarkable recovery with resolution of jaundice and normalisation of liver biochemistry. HBV dna and hepatitis B e antigen were lost from serum subsequently and liver histology had improved at four months. This case report suggests firstly, that advanced FCH can be reversed and secondly, that addition of adefovir dipivoxil to lamivudine and HBIg may be an effective antiviral strategy.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
4/34. A case of coexisting primary biliary cirrhosis and primary sclerosing cholangitis: a new overlap of autoimmune liver diseases.Although the etiology of AIH, PBC, and PSC remains unknown, it is apparent that these autoimmune liver diseases share many common features and can coexist in the same patient. Our patient had features of PBC and later clearly developed a picture of PSC. This case suggests that PBC, PSC, AIH, and autoimmune cholangitis are part of a spectrum of chronic autoimmune liver disease that develop in response to some yet unidentified antigen.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
5/34. Autoimmune hepatitis associated with bile duct injury resembling chronic non-suppurative destructive cholangitis.Autoimmune hepatitis (AIH) and primary biliary cirrhosis (PBC) are representative autoimmune liver diseases in which hepatocytes and intrahepatic bile ducts, respectively, are selectively damaged by autoimmune mechanisms. Bile duct injury and loss is characteristic of PBC and chronic non-suppurative destructive cholangitis (CNSDC), in particular, is a histological hallmark of PBC. In this report, we present an unusual case of AIH accompanied by CNSDC-like bile duct injury in a 46-year-old woman. The patient's serum aminotransferase level was abnormally high. The serum levels of alkaline phosphatase, gamma-GTP and IgG were also elevated, but the IgM level was within normal limits. The titer of antismooth muscle antibody (SMA) was 1:80, while antinuclear autoantibody (ANA) and the M2 fraction of antimitochondrial antibody (AMA) were both negative. Liver biopsy disclosed CNSDC-like bile duct injuries and severe interface hepatitis and lobular hepatitis with perivenular zonal necrosis were observed. The aggregate score of the International Autoimmune Hepatitis Group corresponded to the category of probable AIH. Immunohistochemically, histocompatibility leukocyte antigen-DR, which is aberrantly expressed in the damaged bile ducts of PBC, was not found in the injured bile ducts of this case. Laboratory data were much improved by treatment with prednisone, but ursodeoxycholic acid was not effective. Although the possibility of an overlapping syndrome of AIH- and AMA-negative PBC could not be excluded, this case was diagnosed as AIH with CNSDC-like bile duct lesions.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
6/34. Autoimmune hemolysis associated with primary biliary cirrhosis responding to ursodeoxycholic acid as sole treatment.Coombs' positive autoimmune hemolytic anemia (AIHA) has been rarely described in association with primary biliary cirrhosis (PBC). The previously reported cases have responded to treatment with a combination of corticosteroids and ursodeoxycholic acid (UDCA). We report a case of AIHA occurring in association with PBC, which has responded to treatment with UDCA alone. Possible mechanisms of autoimmune hemolysis in this patient include bile salt induced immune dysregulation and direct damage to red cell membranes by bile salts leading to exposure of neoantigens and development of red cell autoantibodies. A trial of UDCA as a single agent should be considered as initial treatment in this rare disorder.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
7/34. Unusual onset of chronic graft-versus-host disease after adult living-related liver transplantation from a homozygous donor.Graft-versus-host disease (GVHD) is a rare complication that occurs after living-related liver transplantation (LRLT). This condition usually occurs early after transplantation and is fatal. Although one-way matching between a human leukocyte antigen (HLA)-homozygous donor and a haploidentical recipient has been shown to be a significant risk factor for the development of acute GVHD, chronic GVHD has not been clarified. A 50-year-old woman underwent LRLT for primary biliary cirrhosis with her 32-year-old HLA-homozygous son as the donor. The patient developed chronic GVHD 114 days posttransplant, presenting with a skin rash only. Her atypical onset included neither fever nor gastrointestinal symptoms. The patient responded to corticosteroid therapy and is now doing well at home. Careful donor selection and HLA matching before LRLT should be performed to prevent GVHD. However, the risks associated with grafts from homozygous donors may be unavoidable at present because of the shortage of cadaveric donors in japan.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
8/34. Endothelial cell damage in primary biliary cirrhosis: influence of cholestasis and immunological mechanisms.In a study looking for evidence of endothelial cell damage in primary biliary cirrhosis, serum from patients was found to be significantly more cytotoxic to cultured endothelial cells in vitro than normal control serum (P less than 0.001). serum also contained higher levels of von willebrand factor antigen (vWFAg, a specific product of the endothelium) than control serum (P less than 0.001). Cytotoxicity correlated with serum levels of vWFAg (P less than 0.05) and with serum bilirubin (P less than 0.05). No correlations were found between cytotoxicity and circulating immune complexes, c-reactive protein or CH50. The study was controlled by serum from patients with other liver diseases. In these samples vWFAg (P less than 0.003), and bilirubin levels (P less than 0.001) were also raised relative to normal controls. vWFAg levels again correlated with levels of bilirubin (P less than 0.05). Tissue culture experiments showed that purified bilirubin was cytotoxic to human umbilical vein endothelial cells and induced the release of vWFAg. The case report of a patient with obstructive jaundice again indicated that levels of bilirubin and vWFAg were related. These results suggest that endothelial cell damage occurs in both primary biliary cirrhosis and to a lesser extent in other liver diseases, and may be mediated by cholestasis, not by humoral immunological mechanisms.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
9/34. PBC-AIH overlap syndrome with concomitant ITP and Hashimoto's disease with positivity for anti-centromere antibody.We report a case of primary biliary cirrhosis (PBC)-autoimmune hepatitis (AIH) overlap syndrome with concurrent idiopathic thrombocytopenic purpura (ITP) and Hashimoto's disease with positivity for anticentromere antibody. The patient was a 64-year-old woman with symptoms of jaundice and general fatigue. About 30 years earlier, she had been diagnosed as having ITP and had undergone splenectomy. As part of her present history, she had exhibited liver dysfunction in 1995, during the follow-up of Hashimoto's disease, and a liver biopsy led to the diagnosis of PBC. In March 2000, she was admitted to hospital because of general fatigue and jaundice. blood tests revealed: total protein (TP), 6.6 g/dl; gamma-globulin (glb), 35.9%; total bilirubin (T-bil), 9.41 mg/dl; direct bilirubin (D-bil), 7.52 mg/dl; aspartate aminotransferase (AST), 957 U/l; alanine aminotransferase (ALT), 651 U/l; alkaline phosphatase (ALP), 595 U/l; gamma-guanosine triphosphate (GTP), 129 U/l; IgG, 2620 mg/dl; IgM, 223 mg/dl; hepatitis B surface antigen (HBsAg), negative; anti-hepatitis c virus (HCV), negative; antinuclear antibody, positive; antimitchondrial antibody (AMA), negative (by the immunofluorescence [IF] method); and anti-pyruvate dehydrogenase complex (PDC)-E2 antibody, positive (by Western blotting). Anticentromere antibody (ACA), which is an alternative diagnostic marker for PBC, was detected in this patient. prednisolone was administered after admission and liver function test results improved markedly. The liver biopsy in 1995 had revealed infiltration of lymphocytes and plasma cells in the portal areas with fibrous expansion and periportal necrosis. Destructive cholangitis was observed, as well as scattered epitheloid cell granulomas in some portal areas. Liver biopsy after the steroid treatment revealed alleviated necrotic inflammatory responses of hepatocytes, while the destructive cholangitis persisted. This is a very rare case of PBC-AIH overlap syndrome accompanied by ITP and Hashimoto's disease which provides a possible insight into the mechanisms and interplay of autoimmune diseases.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
10/34. A case of primary biliary cirrhosis accompanied with fibrinogen storage disease.We report the first case of primary biliary cirrhosis (PBC) accompanied by fibrinogen storage disease (FSD). A 50-year-old Japanese woman had been treated for numbness of her right-side extremities for 5 years. Mildly elevated serum levels of alkaline phosphatase and gamma-glutamyl transferase were detected. The titers of both anti-mitochondrial (x 320) and anti-mitochondrial M2 (x 84) antibodies were elevated. The biopsied liver specimen showed mononuclear cell infiltrate densely encircling the bile ducts, poorly developed epithelioid cell granuloma, and loss of integrity of bile duct organization, which permitted a diagnosis of stage I PBC according to Scheuer's histologic classification. In addition, round to oval, eosinophilic, homogenous intracytoplasmic inclusions, several microm in average size, with a surrounding halo were found in the vast majority of hepatocytes. These inclusions were negative for the periodic acid-schiff reaction. In immunohistochemistry, the inclusions were positive for fibrinogen and complement c3c, but not for HBs antigen and alpha1-antitrypsin. These findings were identical to FSD. To investigate the mechanism(s) of abnormal fibrinogen storage, immunostaining for heat shock protein 70 and ubiquitin was performed. The former was detected in all intracytoplasmic inclusions, whereas the latter was detected in only some inclusions, suggesting a partial loss of ubiquitin expression.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
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