Chronic liver disease with cholecystosis and inflammation and fibrosis of intrahepatic and extrahepatic bile ducts.
A chronic cholestatic liver disease characterized by inflammation and fibrosis of the intra- and extrahepatic bile ducts which leads to multifocal stricturing of the biliary tree.
The etiology is unknown.
Culminates in cirrhosis and portal hypertension in the majority of patients and is associated with an increased risk of hepatobiliary and colorectal malignancies.
The median age at diagnosis is 40 years of age, and the male to female ratio of patients is 2:1.
Genetic predisposition suggested by increased prevalence of HLA-B6, HLA-DR3 and HLA Drw52a.
Ischemic changes to biliary tree suggested as a cause and related to surgical trauma and vasculitis related to ANCA positive disease.
Ischemic changes to biliary tree suggested as a cause and related to surgical trauma and vasculitis related to ANCA positive disease.
Associated with increased autoantibody levels with antineutrophil cytoplasmic antibodies ANCA in 87% of patients, with anticardiolipin antibodies in 66% of patients, and ANA in 53%.
Prevalence estimated greater than 6 per 100,000 population.
2-4 fold higher prevalence among Jews and in descending order whites, African-Americans, Hispanics and Asians.
75-90% of patients have inflammatory bowel disease, typically, ulcerative colitis (UC).
Of those with inflammatory bowel disease 87% have ulcerative colitis and 13% have Crohn’s disease.
About 4% of patients with irritable bowel syndrome develop this process.
10-20% of patients develop cholangiocarcinoma.
Higher incidence of gallbladder cancer.
Higher rate in Scandinavia.
Males 70%.
Mean age 40 years of age at diagnosis.
A progressive disease ending with cirrhosis and its complications.
Elevation of serum ALP is the most common laboratory abnormality in patients with PSC, although approximately 40% to 50% of patients with Primary sclerosing cholangitis may normalize their ALPs at some point in the course of their disease.
With Primary sclerosing cholangitis serum aminotransferases may be elevated to 2 to 3 times the upper limit of normal.
Serum autoantibodies are nonspecific and are not routinely used to establish a diagnosis of Primary sclerosing cholangitis .
Individuals may be asymptomatic and diagnosis is made when they begin to have symptoms.
MRCP is a noninvasive and an accurate and cost-effective method of detecting PSC.
Patients with small-duct PSC have normal cholangiograms and require liver biopsies to establish the diagnosis of PSC.
There is a wide range of symptoms PSC, ranging from asymptomatic individuals to patients presenting with advanced associated malignancies or hepatic decompensation secondary to portal hypertension.
Cholangiocarcinoma is the most common hepatobiliary malignancy diagnosed in patients with PSC and is the leading cause of mortality.
Patients with PSC are at increased risk of malignancies, including cholangiocarcinoma (CCA), hepatocellular carcinoma (HCC), gallbladder adenocarcinoma (GBCA), and colorectal cancer.
Frequency of diagnosis increased with endoscopic retrograde cholangiopancreatography.
In some studies 20-40% of patients are asymptomatic and this high number relates to the screening of patients with ulcerative colitis for elevated alkaline phosphatase levels and performing ERCP.
Median transplant-free survival ranges from 10 to 21 years after diagnosis.
Patients present with fatigue, pruritus, jaundice and right upper quadrant pain.
Symptoms have a tendency to remit and recur.
Patients occasionally present with acute hepatitis like process.
Recurrent episodes of bacterial cholangitis occur in 10-15% of patients during the course of the illness.
Treatment limited to liver transplantation.
There is no effective medical therapy for PSC.
However, PSC may still recur following liver transplantation in approximately 8.6% to 27% of persons, with a median onset of 4.7 years.
Fourth leading indication for liver transplantation.