Cholestatic Liver Disease: from Pathophysiology to Treatment
1P.J. Safarik University, Kosice, Slovakia
2University of Zagreb, Zagreb, Croatia
3Jagiellonian University, Krakow, Poland
4Charles University, Prague, Czech Republic
Cholestatic Liver Disease: from Pathophysiology to Treatment
Description
Cholestatic diseases are a group of common liver diseases that affect bile ducts of all calibres. According to the place of involvement, cholestasis may be divided into intrahepatic and extrahepatic. The role of subclinical inflammation and bowel microbiota has been recently studied in the pathophysiology of cholestasis. Diagnostic methods (immunopathology, biliary tree volumetry, etc.) have improved and advances have also occurred in the pharmacotherapy of cholestatic diseases.
Primary biliary cholangitis (PBC) causes intrahepatic cholestasis. It is an autoimmune disease where approximately 95% of patients present with AMA-M2 antibodies. Anti sp100 and anti gp210 antibodies are common in AMA-negative PBC. The disease is more common in women, which may be related to estrogen receptor alpha activation. The incidence of PBC is stable, but global prevalence has been rising in recent years, especially in developed countries, which is likely to be related to lifestyle changes. Ursodeoxycholic acid still remains the gold standard of PBC treatment. Add-on treatment with licensed obeticholic acid, or off-label treatment with bezafibrate may be offered to nonresponders to UDCA. Other molecules in the treatment of PBC are also being studied. Primary sclerosing cholangitis (PSC) affects both intra- and extrahepatic biliary ducts. PSC is associated with ulcerative colitis in most patients, although the connection between these two diseases has not yet been precisely explained. Currently, no licensed pharmacotherapy that improves the survival or quality of life of PSC patients is available, but endoscopic treatment of dominant strictures in PSC can improve patient’s survival and also quality of life. Some patients with PSC develop cholangiocarcinoma, which worsens the prognosis of PSC. Despite advances in the diagnosis and treatment of PBC and PSC in recent years, many patients still progress to decompensated liver cirrhosis, where liver transplantation is the only effective therapeutic option. The most common causes of extrahepatic cholestasis are choledocholithiasis, benign and malignant bile duct strictures, pancreatitis, pancreatic tumours, and pseudocysts. Endoscopic methods have a firm place in the diagnosis and treatment of cholestatic diseases of the hepatobiliary tract. The endoscopic retrograde cholangiopancreaticography continues to play a key role, but in recent years the role of the EUS, as well as cholangioscopy, has grown significantly - mainly in the form of direct single operator cholangioscopy.
This Special Issue welcomes scientists to contribute original research articles and clinical studies as well as review articles describing the current state of the art in the field of cholestatic liver diseases. This Special Issue aims to attract both practitioners and researchers in the areas of hepatology, gastroenterology, radiology, and internal medicine.
Potential topics include but are not limited to the following:
- Epidemiology of cholestatic liver diseases
- Molecular aspects of cholestatic liver diseases
- Role of fecal microbiota in cholestatic liver diseases
- Advances in endoscopic treatment of cholestatic liver diseases
- Advances in pharmacotherapy of cholestatic liver diseases
- Prognostic aspects of cholestatic liver diseases
- Perspectives of new diagnostic and therapeutic methods in cholestatic liver diseases