Primary sclerosing cholangitis

  title={Primary sclerosing cholangitis},
  author={Gideon M. Hirschfield and Tom Hemming Karlsen and Keith D. Lindor and David H. Adams},
  journal={The Lancet},

Figures and Tables from this paper

Advances in primary sclerosing cholangitis.
Primary sclerosing cholangitis: an update
Therapies are still lacking to cure the disease or to halt its progression, and unfortunately, primary sclerosing cholangitis can recur after transplantation.
Management of primary sclerosing cholangitis and its complications: an algorithmic approach
PSC pathogenesis is summarized and an algorithmic approach to diagnostic procedures and recommendations for the management of PSC and its complications are provided, including promising treatment options subject to current clinical trials.
Pathogenesis and clinical spectrum of primary sclerosing cholangitis
Despite past and present research, PSC remains an enigmatic biliary disease with few viable treatment options, and liver transplantation is the only known cure for PSC.
Primary Sclerosing Cholangitis
Among the different cancers complicating PSC, CCA is the most relevant, because it is more frequent and because the prognosis is poor (5-year survival <10%).
Cholestatic Liver Injury: Care of Patients With Primary Biliary Cholangitis or Primary Sclerosing Cholangitis.
Patients with PSC should be monitored for evidence of cholangiocarcinoma, colon cancer, and gallbladder polyps as they are at an increased risk of malignant neoplasms.
Differential Diagnosis from Primary Sclerosing Cholangitis
Timely diagnosis of IgG4-SC can lead clinicians to prescribe adequate corticosteroid therapy that can reverse bile duct strictures/wall thickening and cholestatic liver dysfunction and could potentially prevent future advanced liver disease.
Primary sclerosing cholangitis: diagnosis and treatment.
There is no causally directed treatment for primary sclerosing cholangitis and early diagnosis, complication management, and the evaluation of an optimally timed liver transplantation are the main determinants of outcome.
Liver Disease in Pediatric Inflammatory Bowel Disease
This chapter strives to facilitate this task by providing an overview of and suggesting clinical approaches to various hepatobiliary conditions associated with pediatric IBD.


Primary sclerosing cholangitis: Diagnosis and management
Primary sclerosing cholangitis is a chronic cholestatic liver disease caused by progressive inflammatory destruction of intra hepatic and extrahepatic bile ducts, and ultimately cirrhosis, and affects primarily young to middle-aged men.
Dissecting the genetic susceptibility for cholangiocarcinoma in primary sclerosing cholangitis
Primary sclerosing cholangitis (PSC) is an idiopathic cholestatic liver disease caused by fibrosing inflammation of the bile ducts and represents an established risk factor for development of
[Primary sclerosing cholangitis].
During treatment with UDCA stenoses of major ducts may develop and early endoscopic dilatation is highly effective and in patients with endstage disease, UDCA is not effective and liver transplantation is indicated.
Ischemic-Like Cholangiopathy With Secondary Sclerosing Cholangitis in Critically Ill Patients
Ischemic injury of the biliary tree with the formation of biliary casts and subsequent ongoing biliary infection due to multiresistant bacteria seem to be major pathogenic mechanisms in the development of this new entity of sclerosing cholangitis.
Primary sclerosing cholangitis and celiac disease. A novel association.
The association of primary sclerosing cholangitis and celiac disease was observed in three patients, an association not previously reported, and the relationship between the two diseases remains unknown, although an immunologic connection is suspected.
Sclerosing cholangitis: A focus on secondary causes
A comprehensive review of SSC is offered, including a wide variety of other associations have been reported recently, including autoimmune pancreatitis, portal biliopathy, eosinophillic and/or mast cell cholangitis, hepatic inflammatory pseudotumor, recurrent pyogenic cholangsitis, primary immune deficiency, and AIDS‐related cholANGiopathy.
Liver transplantation for primary sclerosing cholangitis: timing, outcome, impact of inflammatory bowel disease and recurrence of disease.
  • R. Wiesner
  • Medicine
    Best practice & research. Clinical gastroenterology
  • 2001
There is an increased incidence of both acute and chronic rejection, hepatic artery thrombosis and biliary stricturing in PSC patients undergoing liver transplantation.
Natural history and prognostic models in primary sclerosing cholangitis.
The development of mathematical models by multivariable regression techniques (most notably Cox proportional hazards regression) has allowed for an improved description of overall survival on an individual basis among patients with PSC.
Primary sclerosing cholangitis: overview and update
Primary sclerosing cholangitis is a chronic cholestatic liver disorder that most commonly affects middle-aged men, and patients with PSC have reduced survival rates compared with the general population.