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*

What are the new contents of the guideline since 2010?A.Patients with primary and non-primary sclerosing cholangitis (PSC) are included in these guidelines for the diagnosis and management of cholangiocarcinoma.B.Define "related stricture" as any biliary or hepatic duct stricture accompanied by the signs or symptoms of obstructive cholestasis and/or bacterial cholangitis.C.Patients who have had an inconclusive report from MRI and cholangiopancreatography should be reexamined by high-quality MRI/cholangiopancreatography for diagnostic purposes. Endoscopic retrograde cholangiopancreatography should be avoided for the diagnosis of PSC.D. Patients with PSC and unknown inflammatory bowel disease (IBD) should undergo diagnostic colonoscopic histological sampling, with follow-up examination every five years until IBD is detected.E. PSC patients with IBD should begin colon cancer monitoring at 15 years of age.F. Individual incidence rates should be interpreted with caution when using the new clinical risk tool for PSC for risk stratification.G. All patients with PSC should be considered for clinical trials; however, if ursodeoxycholic acid (13-23 mg/kg/day) is well tolerated and after 12 months of treatment, alkaline phosphatase (γ- Glutamyltransferase in children) and/or symptoms are significantly improved, it can be considered to continue to be used.H. Endoscopic retrograde cholangiopancreatography with cholangiocytology brushing and fluorescence in situ hybridization analysis should be performed on all patients suspected of having hilar or distal cholangiocarcinoma.I.Patients with PSC and recurrent cholangitis are now included in the new unified network organ sharing policy for the end-stage liver disease model standard.J. Liver transplantation is recommended after neoadjuvant therapy for patients with unresectable hilar cholangiocarcinoma with diameter < 3 cm or combined with PSC and no intrahepatic (extrahepatic) metastases.
Child ; Humans ; Cholangitis, Sclerosing/diagnosis* ; Constriction, Pathologic/complications* ; In Situ Hybridization, Fluorescence ; Cholangiocarcinoma/therapy* ; Liver Diseases/complications* ; Cholestasis ; Inflammatory Bowel Diseases/therapy* ; Bile Ducts, Intrahepatic/pathology* ; Bile Duct Neoplasms/therapy*

Sclerosing cholangitis is a spectrum of chronic progressive cholestatic liver disease characterized by inflammation, fibrosis, and stricture of the bile ducts, which can be classified as primary and secondary sclerosing cholangitis. Primary sclerosing cholangitis is a chronic progressive liver disease of unknown cause. On the other hand, secondary sclerosing cholangitis has identifiable causes that include immunoglobulin G4-related sclerosing disease, recurrent pyogenic cholangitis, ischemic cholangitis, acquired immunodeficiency syndrome-related cholangitis, and eosinophilic cholangitis. In this review, we suggest a systemic approach to the differential diagnosis of sclerosing cholangitis based on the clinical and laboratory findings, as well as the typical imaging features on computed tomography and magnetic resonance (MR) imaging with MR cholangiography. Familiarity with various etiologies of sclerosing cholangitis and awareness of their typical clinical and imaging findings are essential for an accurate diagnosis and appropriate management.
Adult ; Aged ; Aged, 80 and over ; Bile Ducts/*pathology ; Cholangiography/*methods ; Cholangitis/diagnosis/*pathology ; Cholangitis, Sclerosing/*diagnosis/pathology ; Cholestasis/diagnosis/*pathology ; Chronic Disease ; Constriction, Pathologic/diagnosis ; Diagnosis, Differential ; Female ; Humans ; Immunoglobulin G/immunology ; Liver/pathology ; Magnetic Resonance Imaging/methods ; Male ; Middle Aged ; Tomography, X-Ray Computed/methods

BACKGROUND/AIMS: The mortality rate of pyogenic liver abscess (PLA) has decreased dramatically, but it remains a potentially life threatening disease. Most cases are cryptogenic or occur in elderly men with underlying biliary tract disease. Although several studies have addressed the characteristics and etiology of PLA, research on factors affecting PLA-associated mortality is lacking. This study intended to identify the clinical and radiological features, pathogens, complications, and predictors of mortality in Korean PLA patients. METHODS: The medical records of 231 PLA patients diagnosed at Yeungnam University Medical Center between January 2010 and January 2014 were analyzed. A diagnosis of PLA was made based on imaging studies and blood and abscess cultures. The clinical, radiological, and laboratory findings of patients were analyzed. RESULTS: The mean patient age was 64.0±12.9 years and the male to female ratio was 1.5:1. Klebsiella pneumoniae was the predominant organism isolated from hepatic abscesses (69.9%) and blood (74.2%). The most common complication was pleural effusion (35.8%) and most common co-infection was cholangitis (8.2%). The overall mortality rate of PLA was 6.9% (16/231), and was significantly higher in patients with a history of liver abscess (OR 5.970, 95% CI 1.207-29.529; p=0.028), bilirubinemia (>2 mg/dL) (OR 9.541, 95% CI 2.382-38.216; p=0.001), thrombocytopenia (<140×10(3)/µL) (OR 4.396, 95% CI 1.130-17.106; p=0.033), or anemia (<12 g/dL) (OR 13.277, 95% CI 1.476-119.423; p=0.021). CONCLUSIONS: The prognosis of PLA appears to be dependent on underlying pathologies and severity of condition. More aggressive treatment should be considered if a poor prognosis is expected.
Abscess ; Academic Medical Centers ; Aged ; Anemia ; Biliary Tract Diseases ; Cholangitis ; Coinfection ; Diagnosis ; Female ; Humans ; Hyperbilirubinemia ; Klebsiella pneumoniae ; Liver Abscess ; Liver Abscess, Pyogenic* ; Male ; Medical Records ; Mortality* ; Pathology ; Pleural Effusion ; Prognosis ; Retrospective Studies* ; Risk Factors ; Thrombocytopenia

Biliary hamartoma and congenital hepatic fibrosis belong to fibrocystic disorders originating from ductal plate malformation. A 66-year-old man who had incidentally been diagnosed with biliary hamartoma two years ago presented to us with recurrent acute cholangitis. In the first episode, he had presented with septic shock and was treated with endoscopic retrograde cholangiopancreatography (ERCP) and cholecystectomy under the diagnosis of acute cholecystitis and cholangitis. However, during a two-month follow-up period, the patient experienced four episodes of acute cholangitis. Because he showed normal ERCP, and biliary hamartoma is usually asymptomatic, a liver biopsy was performed. Pathology revealed combined features of biliary hamartoma and congenital hepatic fibrosis, characterized as periportal fibrosis and intrahepatic ductular dysplasia. During follow-up for the last six months, he had experienced two episodes of acute cholangitis and was treated with antibiotics. A follow-up abdominal CT scan revealed aggravated hepatosplenomegaly compared to that of two years ago. We report a case of combined congenital hepatic fibrosis and biliary hamartoma and a literature review.
Aged ; Anti-Bacterial Agents ; Biopsy ; Cholangiopancreatography, Endoscopic Retrograde ; Cholangitis* ; Cholecystectomy ; Cholecystitis, Acute ; Diagnosis ; Fibrosis* ; Follow-Up Studies ; Hamartoma* ; Humans ; Liver ; Pathology ; Shock, Septic ; Tomography, X-Ray Computed

IgG4-associated cholangitis can mimic hilar cholangiocarcinoma. Previously reported patients with IgG4-associated cholangitis mimicking cholangiocarcinoma had elevated serum IgG4 levels and long-segment biliary strictures. However, in the absence of other diagnostic criteria for malignancy, IgG4-associated cholangitis should remain a consideration among patients with normal serum IgG4 and a hilar mass suspicious for cholangiocarcinoma. The presence of a hilar mass and a malignant-appearing biliary stricture in two patients with normal serum IgG4 prompted further evaluation and subsequent concomitant liver and bile duct resection and reconstruction. The diagnosis of IgG4-associated cholangitis was established during the pathologic evaluation of the resected specimens. IgG4-associated cholangitis is a known imitator of hilar cholangiocarcinoma and should be considered in the differential diagnosis even among serologically IgG4-negative patients with a hilar mass prior to operative resection.
Aged ; Bile Ducts/pathology/surgery ; Cholangitis/blood/*diagnosis ; Diagnosis, Differential ; Humans ; Immunoglobulin G/*blood ; Klatskin Tumor/blood/*diagnosis ; Liver/pathology/surgery ; Male

Intraductal tumor invasion of hepatocellular carcinoma (HCC) is considered rare. Transarterial chemoembolization (TACE) is effective for tumor thrombus of HCC in the bile duct. However, a few cases of obstructive jaundice caused by migration of a tumor fragment after TACE have recently been reported. The aim of this study was to identify factors that affect tumor migration after TACE. At this writing, a review of the medical literature disclosed seven reported cases of biliary obstruction caused by migration of a necrotic tumor cast after TACE. We, herein, report on an additional case of acute obstructive cholangitis complicated by migration of a necrotic tumor cast after TACE for intrabile duct invasion of HCC, in a 71-year-old man. The tumor cast in the common bile duct was removed successfully using a basket during ERCP and was pathologically confirmed to be a completely necrotic fragment of HCC. The patient's symptoms showed dramatic improvement. In summary, physicians should be aware of acute obstructive cholangitis complicated by tumor migration in a patient undergoing TACE. We suggest that an intrabile duct invasion would be a major predisposing factor of tumor migration after TACE and drainage procedures such as ERCP or percutaneous transbiliary drainage could be effective treatment modalities in these patients.
Acute Disease ; Aged ; Antineoplastic Agents/administration & dosage ; Bile Ducts, Intrahepatic/pathology ; Carcinoma, Hepatocellular/*diagnosis/pathology/therapy ; Chemoembolization, Therapeutic/adverse effects ; Cholangiopancreatography, Endoscopic Retrograde ; Cholangitis/*etiology ; Humans ; Jaundice, Obstructive/etiology ; Liver Neoplasms/*diagnosis/pathology/therapy ; Male ; Necrosis/pathology ; Sphincterotomy, Endoscopic ; Thrombosis/etiology ; Tomography, X-Ray Computed

IgG4-related systemic diseases are characterized by a diffuse or mass forming inflammatory reaction rich in lymphocytes and IgG4-positive plasma cells (lymphoplasmacytic infiltration), fibrosclerosis of variable organs and obliterative phlebitis. They usually involve various organs including the pancreas, bile duct, gallbladder, salivary gland, retroperitoneum, kidney, lung, and prostate. However, most of them are accompanied by autoimmune pancreatitis, and good response to steroid treatment is one of the hallmarks of this disease. We report a case of an 67-year-old man with IgG4 associated sclerosing cholangitis, who was diagnosed by endoscopic retrograde cholangiopancreatography and successfully treated with steroid therapy.
Aged ; Anti-Inflammatory Agents/therapeutic use ; Autoimmune Diseases/complications/diagnosis ; Bile Ducts, Intrahepatic/pathology/ultrasonography ; Cholangiopancreatography, Endoscopic Retrograde ; Cholangitis, Sclerosing/complications/*diagnosis/drug therapy ; Common Bile Duct/pathology/ultrasonography ; Humans ; Immunoglobulin G/*blood ; Immunohistochemistry ; Male ; Pancreatitis/complications/diagnosis ; Prednisolone/therapeutic use ; Tomography, X-Ray Computed

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

We describe an unusual presentation of Clonorchis sinensis infection with obstructive jaundice due to duodenal papillitis which was relieved dramatically by endoscopic sphincterotomy. A 26-yr-old male presented with complaints of fatigue, weight loss and painless jaundice. The history was significant for frequent ingestion of raw freshwater fish. The patient underwent endoscopic retrograde cholangiopancreatography for evaluation of obstructive jaundice. The duodenal papilla was markedly edematous with a bulging configuration and hyperemic changes at the orifice. Cholangiography revealed mild bile duct dilatation and irregular wall changes with multiple indentations. However, there were no biliary stricture or stones noted as the cause of obstructive jaundice. We performed an endoscopic sphincterotomy for effective bile drainage through the duodenal papilla. After the sphincterotomy, the patient's jaundice was dramatically improved. Pathology of the duodenal papilla showed eosinophilic infiltration of the mucosa. Parasitic eggs, consistent with the diagnosis of C. sinensis, were found in the bile sample.
Adult ; *Ampulla of Vater ; Animals ; Anthelmintics/therapeutic use ; Bile/parasitology ; Cholangiopancreatography, Endoscopic Retrograde ; Cholangitis/*diagnosis/parasitology/pathology ; Clonorchiasis/*diagnosis ; Clonorchis sinensis/drug effects/isolation & purification ; Duodenum/pathology ; Humans ; Jaundice, Obstructive/*diagnosis/etiology ; Male ; Praziquantel/therapeutic use ; Sphincterotomy, Endoscopic ; Tomography, X-Ray Computed