Complications
Most patients with primary sclerosing cholangitis will progress to cirrhosis and develop complications of end-stage liver disease including oesophageal varices, ascites, and hepatic encephalopathy.
The management of these complications is similar to that for advanced, chronic liver disease from other causes.
Most patients with primary sclerosing cholangitis will progress to cirrhosis and develop complications of end-stage liver disease including oesophageal varices, ascites, and hepatic encephalopathy.
The management of these complications is similar to that for advanced, chronic liver disease from other causes.
Most patients with primary sclerosing cholangitis will progress to cirrhosis and develop complications of end-stage liver disease including oesophageal varices, ascites, and hepatic encephalopathy.
The management of these complications is similar to that for advanced, chronic liver disease from other causes.
Most patients with primary sclerosing cholangitis will progress to cirrhosis and develop complications of end-stage liver disease including oesophageal varices, ascites, and hepatic encephalopathy.
The management of these complications is similar to that for advanced, chronic liver disease from other causes.
Increased risk in patients with cirrhosis (comparable to the risk in cirrhosis from other cholestatic liver diseases).
Prevalence around 2%.[103]
Screening patients with cirrhosis is recommended with 6-monthly ultrasound and serum alpha-fetoprotein measurement.[2][104][105]
Liver transplantation should be considered for patients with primary sclerosing cholangitis (PSC) and hepatocellular carcinoma according to standard guidelines.[2][3]
Routine surveillance for hepatobiliary and colonic malignancy is not suggested for children with PSC.[2]
Patients with primary sclerosing cholangitis (PSC) have an up to 20% lifetime risk of developing cholangiocarcinoma.[106] Patients with PSC are 160-400 times more likely to develop cholangiocarcinoma than the general population.[18][107][108]
Cholangiocarcinoma is a relatively common complication that can be present at diagnosis of PSC or arise at any stage of the disease.[103][109] Delayed diagnosis often results in its discovery at an advanced stage when it cannot be resected.[3]
Should be suspected in patients with newly diagnosed PSC who have a high-grade stricture, those who present with rapidly progressing jaundice, weight loss, or abdominal pain, or those who have a new mass lesion identified on imaging.[2]
Whether to routinely screen for cholangiocarcinoma in people with PSC is an area of debate.[104][110] Guidelines suggest yearly surveillance for cholangiocarcinoma and gallbladder malignancy in all patients with large-duct PSC.[2][3][104] Routine surveillance for hepatobiliary and colonic malignancy is not suggested for children with PSC.[2][3]
Patients should be screened for reductions in bone mineral density at the time of primary sclerosing cholangitis diagnosis and at intervals of 2 to 3 or 4 years (based on risk factors) using dual energy x-ray absorptiometry.[2]
People who are diagnosed with osteoporosis should receive treatment. A referral to Endocrinology should be considered.
Vitamin A, D, E, and K deficiencies occur in 2% to 40% of patients with primary sclerosing cholangitis.[111]
Can be screened for by measurement of fat-soluble vitamin levels and the prothrombin time.
Established deficiencies should be treated.
Increased risk after endoscopic procedures, but can develop spontaneously.
Commonly caused by aerobic enteric organisms (e.g., Escherichia coli, Klebsiella species, Enterococcus faecalis).
Broad-spectrum antibiotics should be initiated promptly.
Long-term antibiotics may be helpful in patients with frequent, recurrent bouts.
Treatment of dominant/relevant stricture (if present) should be considered.
Liver transplantation should be considered for recurrent bacterial cholangitis and/or severe pruritus or jaundice despite endoscopic or medical therapy.[2]
Biliary strictures predispose to bile stasis and intraductal stone formation.
Endoscopic retrograde cholangiopancreatography for evaluation and extraction of obstructing stones is indicated in patients with worsening cholestasis or jaundice.
Incidental stones do not need to be treated.
Increased risk of gallbladder carcinoma in patients with primary sclerosing cholangitis (PSC).[103]
Gallbladder polyps should prompt cholecystectomy if they are 8 mm in size or bigger (European guidelines) or bigger than 8 mm (US guidance).[2][3]
European guidelines also recommend cholecystectomy for smaller polys that are growing in size, whereas US guidance suggests polyps 8 mm in size or smaller may be monitored by ultrasound every 6 months.[2][3]
Screening for gallbladder polyps with abdominal ultrasound is recommended by some.[112]
Guidelines suggest yearly surveillance for cholangiocarcinoma and gallbladder malignancy in all patients with large-duct PSC.[2][3][104]
Routine surveillance for hepatobiliary and colonic malignancy is not suggested for children with PSC.[2]
Patients with concurrent primary sclerosing cholangitis (PSC) and ulcerative colitis are at increased risk for the development of colonic neoplasia (dysplasia or carcinoma) compared with patients with ulcerative colitis alone.[113]
Cumulative incidence of 7%.[103]
Patients with PSC should undergo a colonoscopy at the time of diagnosis of PSC to screen for colitis (even if they are asymptomatic), and it should be repeated (US guidance) or considered (European guidelines) every 5 years, or whenever patients have symptoms suggestive of inflammatory bowel disease.[2][3]
Patients with PSC and established inflammatory bowel disease should undergo surveillance colonoscopy yearly from the time of diagnosis of colitis (4 quadrant biopsies every 10 cm with additional targeted biopsies of abnormal areas).
Routine surveillance for hepatobiliary and colonic malignancy is not suggested for children with PSC.[2]
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