Despite considerable advances over the last 2 decades in the molecular understanding of cholestasis and cholestatic liver disease, little improvement has been made in diagnostic tools and therapeutic strategies.
Professor Boberg and colleagues critically reviewed controversial aspects of the scientific basis for common clinical practice in primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC) and to discuss key ongoing challenges to improve patient management.
The researchers performed a literature search using PubMed and by examining the reference lists of relevant review articles related to the clinical management of PBC and PSC.
|Timing of liver transplantation will benefit from better appreciation of pre-transplant disease mechanisms|
|Alimentary Pharmacology & Therapeutics|
The research team considered articles on the background of the European Association for the Study of the Liver (EASL) and the American Association for the Study of Liver Diseases (AASLD) practice guidelines and clinical experience of the authors.
Ongoing challenges in PBC mainly pertain to the improvement of medical therapy, particularly for patients with a suboptimal response to ursodeoxycholic acid.
In PSC, development of medical therapies and sensitive screening protocols for cholangiocarcinoma represent areas of intense research.
To rationally improve patient management, a better understanding of pathogenesis, including complications like pruritis and fatigue, is needed and there is a need to identify biomarker end-points for treatment effect and prognosis.
The team note that timing of liver transplantation and determining optimal regimens of immunosuppression post-liver transplantation will also benefit from better appreciation of pre-transplant disease mechanisms.
Dr Boberg's team concludes, "Controversies in the management of PBC and PSC relate to topics where evidence for current practice is weak and further research is needed."