Complications
- The complications common to all of the chronic cholestatic liver diseases, such as PSC and PBC, include fatigue, pruritus, steatorrhea, fat-soluble vitamin deficiencies (A, D, E, and K), metabolic bone disease, hypercholesterolemia xanthomas, hypothyroidism, and anemia. There is a reported association of PBC with Sjögren syndrome, Raynaud phenomenon, and sicca symptoms.
- Approximately 20% of patients with PSC develop a dominant stricture in the intrahepatic or extrahepatic biliary tree. Medical therapy to treat biliary strictures has been ineffective. Nonsurgical modalities to relieve biliary obstruction, such as endoscopically- or radiologically–guided balloon dilation of strictures or placement of prosthetic stents across strictures, should be attempted initially.
- Choledocholithiasis and cholelithiasis due to cholesterol and/or pigment stones may be present in up to one third of patients with PSC. Bacterial cholangitis can occur in patients with PSC.
- Cholangiocarcinoma eventually develops in about 20% of patients with PSC, principally late in the course of long-standing ulcerative colitis and the cirrhotic stage of biliary disease. This complication is difficult to detect, as evidenced by the finding of cholangiocarcinoma in 10% of patients undergoing liver transplantation for PSC.[14]
- The incidence of hepatocellular carcinoma is increased in patients with PBC who have had stage IV disease for many years.[15]
- Patients with both PSC and ulcerative colitis have an increased risk of colon cancer and progression of neoplastic transformation.
Prognosis
- In PSC, several factors suggest a high risk of death. These include advancing age, serum bilirubin, blood hemoglobin, presence or absence of inflammatory bowel disease, and histologic stage on liver biopsy.
Patient Education
- For excellent patient education resources, visit eMedicine's Liver, Gallbladder, and Pancreas Center and Cholesterol Center. Also, see eMedicine's patient education articles Gallstones and Cirrhosis.
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