Aged ; Bile Ducts ; pathology ; Cholangitis ; complications ; Humans ; Jaundice ; complications ; Male

Drug-induced bile duct injury is a specific kind of drug-induced liver injury that has two main pathological types, namely ductopenia, or vanishing bile duct syndrome, and secondary sclerosing cholangitis. However, in recent years, the reports of new drugs that cause bile duct injury have been constantly increasing, and these drugs have different clinicopathological features and a novel pathogenesis. Therefore, this paper summarizes and analyzes the progress and challenges in the etiology, pathogenesis, diagnosis and treatment, and other aspects of drug-induced bile duct injury.
Humans ; Cholestasis/chemically induced* ; Cholangitis, Sclerosing/diagnosis* ; Chemical and Drug Induced Liver Injury/pathology* ; Bile Ducts/pathology*

Autoimmune cholangitis is a clinical constellation of chronic cholestasis, histological changes of chronic nonsuppurative cholangitis and the presence of autoantibodies other than antimitochondrial antibody (AMA). It is uncertain whether this entity is definitely different from AMA positive primary biliary cirrhosis (PBC), though it shows some differences. We report a case of autoimmune cholangitis in a 59-year-old woman, who had been previously diagnosed as AMA-positive PBC associated with rheumatoid arthritis, has been converted to an AMA-negative and anticentromere antibody-positive PBC during follow-up. The response to ursodeoxycholic acid treatment is poor except within the first few months, but prednisolone was dropping the biochemical laboratory data.
Autoantibodies/immunology* ; Case Report ; Cholangitis/pathology ; Cholangitis/immunology* ; Female ; Human ; Liver Cirrhosis, Biliary/pathology ; Liver Cirrhosis, Biliary/immunology* ; Middle Age ; Mitochondria/immunology*

Adult ; Chemoembolization, Therapeutic ; adverse effects ; Cholangitis, Sclerosing ; diagnosis ; etiology ; Humans ; Liver ; pathology ; Male ; Young Adult

No abstract available.
Adult ; Cholangitis, Sclerosing/*diagnosis/pathology ; Diagnosis, Differential ; Humans ; Liver Cirrhosis, Biliary/diagnosis ; Male

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

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

No abstract available.
Aged ; Biopsy, Fine-Needle ; Cholangitis, Sclerosing/*pathology/ultrasonography ; Humans ; Immunoglobulin G/*blood ; Liver/*pathology ; Male ; Tomography, X-Ray Computed

Bile Duct Neoplasms ; etiology ; pathology ; Bile Ducts, Intrahepatic ; pathology ; Carcinoma in Situ ; etiology ; pathology ; Carcinoma, Papillary ; pathology ; Cholangitis, Sclerosing ; complications ; Diagnosis, Differential ; Humans ; Precancerous Conditions ; etiology ; pathology

OBJECTIVETo characterize the clinical, laboratory, imaging and pathological features of primary sclerosing cholangitis (PSC) and investigate the impact of ursodeoxycholic acid (UDCA) therapy on patient prognosis.

METHODSThe medical records of 22 patients diagnosed with PSC between 2002 and 2011 were retrospectively reviewed. The PSC diagnosis had been made in patients with suspect biochemical abnormalities following evaluation by magnetic resonance cholangiopancreatography (MRCP) and/or endoscopic retrograde cholangiopancreatography (ERCP) and percutaneous transhepatic cholangiography (PTC). Fibrosis and inflammation were assessed by immunohistochemical analyses of tissue biopsies. Outcome of patients treated with UDCA (13-15 mg/kg/day, oral) were compared to that of patients without UDCA treatment by the X2 or corrected X2 tests.

RESULTSAmong the 22 PSC patients, the majority was male (n=15) and presented with fatigue, dark urine, and body weight loss (n=15). Four cases had ulcerative colitis. At admission, all 22 cases showed elevated levels of alkaline phosphatase[ALP: (348+/-184) U/L], 19 cases showed elevated alanine aminotransferase [ALT: (94.0+/-67.0) U/L] and aspartate aminotransferase [AST: (98.0+/-67.0) U/L], and 15 cases showed elevated levels of total bilirubin (99.0+/-115.0) mumol/L and direct bilirubin (74.4+/-92.4 mumol/L. ERCP examination showed segmental intrahepatic bile duct stenosis with expansion, and stiff and enlarged gallbladder bile ducts, but unclear findings for the common bile ducts and pancreatic ducts. MRCP showed beading of the intrahepatic bile duct, stiffness of the bile duct wall, and dilation of the common bile duct. Fibrosis and inflammation were observed in the bile ducts, along with hyperplasia and the typical features of "onion skin" fibrosis and fibrous obliterative cholangitis. Five of the 10 patients treated with UDCA improved, and seven of the 12 patients in the non-UDCA treatment group improved. There was no statistically significant difference in outcome between the groups (paired X2=0.333, corrected X2=0.083, P more than 0.05).

CONCLUSIONPSC patients were predominantly male and the common clinical manifestations were fatigue, dark urine, and body weight loss. At admission, serum biochemical indicators of cholangitis were increased significantly and subsequent imaging studies confirmed the suspected diagnosis by showing obvious characteristic changes. UDCA treatment did not significantly improve patient prognosis.

Adult ; Cholangiography ; methods ; Cholangiopancreatography, Endoscopic Retrograde ; Cholangitis, Sclerosing ; diagnostic imaging ; pathology ; Female ; Humans ; Male ; Middle Aged ; Retrospective Studies ; Young Adult