Cholangitis/therapy* ; Humans ; Liver Cirrhosis, Biliary/therapy*

Humans ; Liver Cirrhosis, Biliary ; diagnosis ; therapy

Humans ; Liver Cirrhosis, Biliary ; diagnosis ; etiology ; therapy

In 2015, the Chinese Society of Hepatology and Chinese Society of Gastroenterology issued a consensus on the diagnosis and management of primary biliary cholangitis (PBC). In the past years, more clinical studies have been reported in the field of PBC. To provide guidance to the clinical diagnosis and management of patients with PBC, the Chinese Society of Hepatology invited a panel of experts to assess the new clinical evidence and formulated the current guidelines which comprises 26 clinical recommendations.
Cholangitis/therapy* ; Consensus ; Gastroenterology ; Humans ; Liver Cirrhosis, Biliary/therapy*

Humans ; Liver Cirrhosis, Biliary ; drug therapy ; Ursodeoxycholic Acid ; therapeutic use

Cholangitis, Sclerosing ; diagnosis ; therapy ; Hepatitis, Autoimmune ; diagnosis ; therapy ; Humans ; Liver Cirrhosis, Biliary ; diagnosis ; therapy

Cholangitis, Sclerosing ; complications ; diagnosis ; therapy ; Hepatitis, Autoimmune ; complications ; diagnosis ; therapy ; Humans ; Liver Cirrhosis, Biliary ; complications ; diagnosis ; therapy ; Syndrome

With recent developments, biologic therapies has shown superior efficacy for rheumatic diseases compared with preexisting pharmacologic therapies, which are associated with high costs, non-response in certain patient groups, and severe adverse effects such as infections limiting their wide-spread use and revealing a need for the development of novel treatments. Since discovering the role of bile acid receptors in regulating inflammation, clinical trials evaluating the use of bile acid receptor agonists as a means to potentially treat various inflammatory disorders, such as alcoholic hepatitis, non-alcoholic steatohepatitis, primary biliary cirrhosis, primary sclerosing cholangitis have been ongoing. This review summarizes the results of studies on the anti-inflammatory effects and mechanisms of bile acid receptors and the results of previous to date looking at the use of bile acid receptor agonists in animal models of inflammatory disorders and clinical trials. Furthermore, we present the potentials of the bile acid receptor agonists in the treatment of inflammatory rheumatic diseases, including rheumatoid arthritis.
Arthritis, Rheumatoid ; Bile* ; Biological Therapy ; Cholangitis, Sclerosing ; Fatty Liver ; Hepatitis, Alcoholic ; Humans ; Inflammation ; Liver Cirrhosis, Biliary ; Models, Animal ; Rheumatic Diseases

OBJECTIVEIn order to provide a reliable basis for the diagnosis and treatment of autoimmune hepatitis (AIH) and its overlap syndrome, we investigated the clinical, immunological characteristics of and the therapeutic methods for AIH and AIH-primary biliary cirrhosis (PBC) overlap syndrome.

METHODSOne hundred seven patients (77 with AIH and 30 with AIH-PBC overlap syndrome) were enrolled in the study. Their clinical manifestations, serum liver function tests (LFTs) findings, serum immunoglobulins, liver histopathological changes and their responsiveness to the therapies were investigated.

RESULTSThe age distribution of AIH patients showed a single peak during their fifties and their main clinical manifestations were malaise, abdominal distension, anorexia and jaundice. Serum gamma globulin and IgG were significantly higher than their normal levels. 74% of the patients were positive for anti-nuclear antibody (ANA), 32% of the patients were positive for anti-smooth muscle antibody (AMA), and over 50% of the patients suffered from concurrent extrahepatic autoimmune diseases. The main histological changes in the liver biopsies were interface hepatitis (65%), lobular hepatitis and rosette formation of liver cells. Bridging necrosis was observed in severe AIH cases. In the AIH-PBC overlap syndrome patients, the levels of serum ALT, AST, GGT, ALP and incidences of ANA and AMA/AMA-M2 were all significantly higher than those of the AIH group. After treating AIH patients with prednisolone and azathioprine (Aza), complete response was seen in 42 cases (70%), sustained response was seen in 26 cases (43%). Sixteen cases had relapses after the withdrawal of the treatment or prednisolone dosage was reduced lower than 10 mg/d. The cases having normal serum ALT, AST, gamma-globulin and IgG levels after treatment were still responding to the reduced prednisolone dosage of 5-10 mg/d without azathioprine added. After combination with ursodeoxycholic acid (UDCA) treatment, the liver function tests (AST, ALT, TBil) of AIH-PBC overlap syndrome patients also significantly improved compared to those before the treatment (P<0.01).

CONCLUSIONAIH and AIH-PBC overlap syndrome are not rare in our clinics. Their diagnoses should be based on the clinical presentations, biochemical and immunological indices and liver histological changes. In AIH cases, once their AST, ALT, gamma-globulin and IgG levels return to normal, the prednisolone dosage can be maintained at 5-10 mg/d and Aza can even be withdrawn. Good improvement for patients with AIH-PBC overlap syndrome can be obtained with UDCA and immunosuppression treatment.

Female ; Hepatitis, Autoimmune ; diagnosis ; drug therapy ; Humans ; Liver Cirrhosis, Biliary ; diagnosis ; drug therapy ; Male ; Middle Aged ; Prognosis ; Syndrome

OBJECTIVEEndogenous hydrophobic bile acids may be a pathogenetic factor of biliary complications after orthotopic liver transplantation (OLT).This study was designed to investigate the effects of hydrophilic ursodeoxycholic acid (UDCA), when administered early after OLT, on serum liver tests and on the incidence of biliary complications.

METHODSA total of 112 adult patients undergoing OLT were randomly assigned to one of two groups for receipt of UDCA (13 to 15 mg/kg/d for 4 weeks, n=56) or a placebo (n=56). All patients underwent serum liver testing and measurement of serum bile acids during the 4 weeks following OLT.Patients with T-tube underwent measurement of biliary bile acids during the 4 weeks following OLT.Biliary complications, as well as patient and graft survival rates, were analyzed during the follow-up period (mean of 65.6 months).

RESULTSAt post-OLT days 7, 21 and 28, the UDCA-treated patients showed significantly lower levels of alanine aminotransferase, aspartate aminotransferase and gamma glutamyl transpeptidase (all P less than 0.05).In addition, the UDCA-treated patients showed significantly lower incidence of biliary sludge and casts within the first year post-OLT (3.6% vs.14.3%; x2=3.953, P=0.047). However, there were no significant differences for the incidence of other biliary complications at post-OLT years 1, 3 and 5.The graft and patient survival rates were also similar between the two groups.

CONCLUSIONUDCA, when administered early after OLT, improves results from serum liver tests and decreases the incidence of biliary sludge and casts within the first postoperative year.

Alanine Transaminase ; Aspartate Aminotransferases ; Bile ; Bile Acids and Salts ; Biliary Tract Diseases ; drug therapy ; physiopathology ; Humans ; Liver ; physiopathology ; Liver Cirrhosis, Biliary ; Liver Function Tests ; Liver Transplantation ; Postoperative Complications ; physiopathology ; Ursodeoxycholic Acid ; therapeutic use ; gamma-Glutamyltransferase