Cholangitis, Sclerosing ; diagnosis ; therapy ; Humans

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

Liver transplantation for treating cholangiocarcinoma (CCA) has potential benefits in terms of accomplishing radical resection even in patients with unresectable tumor and treating underlying disease such as primary sclerosing cholangitis (PSC). Despite this ideal rationale, the initial results of liver transplantation alone for CCA have been uniformly poor. Because of a high recurrence rate and a poor survival rate, CCA has been viewed as an absolute contraindication for transplantation. However, from the 1990s, liver transplantation following high dose neoadjuvant radiotherapy with chemotherapy has achieved excellent results, in a few medical centers, for selected patients with unresectable hilar CCA arising in the setting of PSC. Especially, the Mayo Clinic reported a 5-year survival rate of 73% for 120 patients in 2010 by performing neoadjuvant therapy and liver transplantation. We reviewed the results of liver transplantation in patients with hilar CCA and the protocol of the Mayo Clinic to consider the feasibility of liver transplantation for Korean patients with hilar CCA. Although liver transplantation with neoadjuvant therapy cannot be applied to most Korean patients due to the different disease entities and the good results with performing radical surgical resection, it can be considered as an alternative curative treatment option for selected patients with anatomically unresectable hilar CCA or CCA arising in the setting of PSC.
Cholangiocarcinoma ; Cholangitis, Sclerosing ; Humans ; Liver ; Liver Transplantation ; Neoadjuvant Therapy ; Recurrence ; Survival Rate ; Transplants

OBJECTIVETo elucidate clinical and pathological features of primary sclerosing cholangitis (PSC) in order to improve clinician's awareness of this rare disease.

METHODSWe retrospectively analyzed clinical data and follow-up information of 27 PSC patients who were admitted to Beijing Friendship Hospital from January 1990 to November 2009. The patients were classified into classic PSC and small-duct PSC according to biochemistry and imaging results. After 3 to 6 months of therapy, those patients with serum ALT < or = 1.5, TBil < or = 2 and ALP < or = 2.5 ULN were determined as good responders. The treatment results between the two groups were compared.

RESULTS9 out of 27 cases of PSC were small duct PSC and 18 cases were large bile duct or classic PSC. Male patients (7) were less than females (20) and the average age was 47.6 years. Main clinical symptoms included jaundice (85.2%), pruritis (48.1%),fatigue (68.4 %), abdominal pain (40.7%) and fever (14.8%), main physical sign included hepatomegaly (44.4%), splenomegaly (48.1 %) and ascites (14.8%). Laboratory features included elevated IgG (81.8%), positive ANA (69.6%) and pANCA (52.9%). 22% of these PSC patients had ulcerative colitis or Sjogren's syndrome. A small percentage of patients were responsive to standard therapy, of which small duct PSC had a better response than classic PSC (66.7 % vs 33.3%, P = 0.041).

CONCLUSIONSUlcerative colitis (22.2%) is not as common as reported by western countries. Small duct PSC has a better treatment response. Searching of effective treatment regimen for large bile duct PSC is warranted in future studies.

Adolescent ; Adult ; Aged ; Cholangitis, Sclerosing ; pathology ; therapy ; Female ; Humans ; Male ; Middle Aged ; Retrospective Studies ; Young Adult

In 2015, the first Chinese consensus on the diagnosis and management of primary sclerosing cholangitis was issued. In the past years, more data have emerged from the literature. Herein, the Autoimmune Liver Disease Group of the Chinese Society of Hepatology organized an expert group to review the evidence and updated the recommendations to formulate the guidelines. There are 21 recommendations on PSC clinical practice. To facilitate the differentiation between PSC and IgG4-related sclerosing cholangitis, 10 recommendations on IgG4-SC are also attached. These guidelines aim to provide a working reference for the management of PSC and IgG4-SC.
Autoimmune Diseases/diagnosis* ; Cholangitis, Sclerosing/therapy* ; Diagnosis, Differential ; Humans ; Immunoglobulin G

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

Autoimmune Diseases ; drug therapy ; pathology ; Cholangitis ; drug therapy ; immunology ; pathology ; Cholangitis, Sclerosing ; drug therapy ; immunology ; pathology ; Hepatitis, Autoimmune ; drug therapy ; immunology ; pathology ; Humans ; Interferon-gamma ; therapeutic use ; Liver Cirrhosis, Biliary ; drug therapy ; immunology ; pathology ; Ursodeoxycholic Acid ; therapeutic use

Cholangitis, Sclerosing ; diagnosis ; therapy ; Hepatitis, Autoimmune ; diagnosis ; therapy ; Humans ; Liver Cirrhosis, Biliary ; diagnosis ; therapy ; Magnetic Resonance Imaging ; Tomography, X-Ray Computed ; Ultrasonography

Primary sclerosing cholangitis (PSC), a rare progressive liver disease characterized by cholestasis and bile duct fibrosis, has no accepted, effective therapy known to delay or arrest its progression. We report a 15 year old female patient diagnosed with PSC and moderate chronic active ulcerative colitis (UC) who achieved normalization of her liver enzymes and bile ducts, and resolution of her UC symptoms with colonic mucosal healing, after treatment with a single drug therapy of the antibiotic oral vancomycin. We postulate that the oral vancomycin may be acting both as an antibiotic by altering the intestinal microbiome and as an immunomodulator. Oral vancomycin may be a promising treatment for PSC that needs to be further studied in randomized trials.
Bile Ducts ; Child* ; Cholangitis, Sclerosing* ; Cholestasis ; Colitis, Ulcerative* ; Colon ; Drug Therapy ; Female ; Fibrosis ; Gastrointestinal Microbiome ; Humans ; Liver ; Liver Diseases ; Ulcer* ; Vancomycin*