Complications
Most patients with PSC will progress to cirrhosis and develop complications of end-stage liver disease including esophageal 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 PSC will progress to cirrhosis and develop complications of end-stage liver disease including esophageal 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 PSC will progress to cirrhosis and develop complications of end-stage liver disease including esophageal 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 PSC will progress to cirrhosis and develop complications of end-stage liver disease including esophageal 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%.[105]
Screening patients with cirrhosis is recommended with every 6-month ultrasound, computed tomography (CT), or magnetic resonance imaging (MRI), with or without serum alpha-fetoprotein.[2][106][107]
Liver transplantation should be considered for patients with 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 PSC have an up to 20% lifetime risk of developing cholangiocarcinoma.[108] Patients with PSC are 160-400 times more likely to develop cholangiocarcinoma than the general population.[19][109][110]
Cholangiocarcinoma is a relatively common complication that can be present at diagnosis of PSC or arise at any stage of the disease.[105][111] 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]
More commonly develops as an infiltrating stenotic lesion rather than a discrete mass. Differentiation from a benign dominant stricture is challenging; cross-sectional imaging and cholangiographic findings are often indistinguishable from PSC without cholangiocarcinoma. If detected at an earlier stage, PSC-associated malignancy can be treated with a curative surgical intervention.[3]
The recommended initial diagnostic test is contrast-enhanced cross-sectional imaging, and endoscopic retrograde cholangiopancreatography (ERCP) with bile duct brushing and biopsies may follow, as necessary, for diagnosis and staging.[111][112] Serum CA 19-9 can also be assessed if cholangiocarcinoma is suspected, and fluorescence in situ hybridization (FISH), or equivalent, can be considered if brush cytology and histology findings are equivocal.[2][113][114] The European and US guidelines recommend annual abdominal imaging, preferably with magnetic resonance imaging/magnetic resonance cholangiopancreatography (MRI/MRCP), to be considered for cholangiocarcinoma surveillance.[2][3]
Cytology and fluorescence in situ hybridization of ERCP biliary brushings should be performed for all patients with suspected perihilar or distal cholangiocarcinoma.[3] Referral to a specialist center is recommended. Therapeutic options include liver resection, irradiation, brachytherapy or systemic therapy (or combinations), or liver transplantation; however, potentially curative surgical resection is rarely feasible due to the extent of tumor and/or advanced liver disease.[2] Liver transplantation can also be considered in patients with PSC and high-grade biliary dysplasia that has been confirmed by ductal histology or cytology.[2]
Whether to routinely screen for cholangiocarcinoma in people with PSC is an area of debate.[106][115] European guidelines and US guidance suggest yearly surveillance for cholangiocarcinoma and gallbladder malignancy in all patients with large-duct PSC, but carbohydrate antigen 19-9 (CA 19-9) is insufficiently accurate to be suggested for surveillance purposes.[2][3] 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 PSC diagnosis and at intervals of 2 to 3 or 4 years (based on risk factors) using dual energy x-ray absorptiometry (DEXA).[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 PSC.[116]
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.
Aerobic enteric organisms most common ( 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.
Magnetic resonance cholangiopancreatography (MRCP) should be considered to rule out the presence of any relevant strictures.[3]
If bacterial cholangitis does not adequately respond to antibiotics, endoscopic retrograde cholangiopancreatography should be performed.[2][3]
Treatment of dominant 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 PSC.[105]
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.[117]
US guidance suggests yearly surveillance for cholangiocarcinoma and gallbladder malignancy in all patients with large-duct PSC.[3]
Routine surveillance for hepatobiliary and colonic malignancy is not suggested for children with PSC.[2]
Patients with concurrent PSC and ulcerative colitis are at increased risk for the development of colonic neoplasia (dysplasia or carcinoma) compared with patients with ulcerative colitis alone.[118]
Cumulative incidence of 7%.[105]
Treatment with ursodiol may be protective.[119][120]
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][121]
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).
High-grade dysplasia, dysplasia-associated lesions or masses (referred to as DALM), and carcinoma are indications for colectomy.
Colectomy is also recommended if, despite optimal medical therapy, symptomatic colonic inflammatory activity persists.[2] Low-grade dysplasia can be managed with ongoing surveillance, or colectomy if it is present on multiple occasions or at multiple locations.[2]
Routine surveillance for hepatobiliary and colonic malignancy is not suggested for children with PSC.[2]
Use of this content is subject to our disclaimer