Which of the following statements about primary sclerosing cholangitis are correct?

Primary Sclerosing Cholangitis: Correct Statements

The correct answers are (c) and (e): PSC often coexists with inflammatory bowel disease, and patients have a high risk of developing cholangiocarcinoma.

Analysis of Each Statement

Statement A: Diagnosis based on antimitochondrial antibodies - INCORRECT

• Antimitochondrial antibodies (AMA) are NOT used to diagnose PSC—they are characteristic of primary biliary cholangitis (PBC), not PSC 1
• PSC diagnosis relies on characteristic bile duct changes on MRCP (magnetic resonance cholangiopancreatography) as the primary imaging modality 1, 2
• This is a common pitfall: confusing PSC with PBC, which are distinct cholestatic liver diseases with different autoantibody profiles

Statement B: Disease rarely leads to liver failure - INCORRECT

• PSC is a progressive disease that commonly leads to end-stage liver failure 3, 4, 2
• Progressive biliary and hepatic damage results in portal hypertension and hepatic failure in a significant majority of patients over a 10-15 year period from initial diagnosis 3
• Liver transplantation is a well-recognized and necessary treatment for PSC, with eligibility assessed according to national guidelines 1
• PSC is associated with 4-fold increased mortality compared to age- and sex-matched populations 3

Statement C: Often coexists with inflammatory bowel disease - CORRECT

• PSC is strongly associated with IBD, occurring in approximately 70-88% of patients 5, 3, 2
• The association is particularly strong with ulcerative colitis (1.4% prevalence) compared to Crohn's disease (0.13% prevalence) 6
• All patients with PSC should be screened for colitis using colonoscopy and colonic biopsies 1
• Patients with PSC-IBD require annual colonoscopic surveillance from the time of colitis diagnosis due to markedly increased colorectal cancer risk 1
• Concomitant PSC confers additional risk for colorectal cancer beyond IBD alone, with an increased absolute risk of up to 31% 1

Statement D: Maintenance therapy with low-dose steroids - INCORRECT

• Corticosteroids and immunosuppressants are NOT indicated for treatment of classic PSC 1, 7
• Steroids may only be indicated in patients with additional features of autoimmune hepatitis (AIH) or IgG4-related sclerosing cholangitis (IgG4-SC), which are distinct conditions 1
• There are no proven medical therapies that alter the natural course of PSC 3, 4
• Ursodeoxycholic acid (UDCA) is NOT recommended for routine treatment and does not prevent colorectal cancer or cholangiocarcinoma 1, 7

Statement E: High risk of cholangiocarcinoma - CORRECT

• PSC confers a markedly increased risk of hepatobiliary cancers, particularly cholangiocarcinoma and gallbladder cancer 3, 4
• Cancer is the leading cause of mortality in patients with PSC 3
• Among Asian patients with PSC-IBD, 7.2% developed cholangiocarcinoma 6
• When cholangiocarcinoma is clinically suspected, referral for specialist multidisciplinary meeting review is essential 1
• Annual ultrasound surveillance of the gallbladder should be performed in PSC patients 1

Key Clinical Pitfalls to Avoid

• Do not confuse PSC with PBC: PSC lacks antimitochondrial antibodies and has different imaging findings 1
• Do not underestimate disease severity: PSC is progressive and commonly leads to liver failure, not a benign condition 3, 4
• Do not use steroids routinely: They are contraindicated in classic PSC unless overlap syndromes are present 1, 7
• Do not miss IBD screening: All PSC patients need colonoscopy to assess for coexistent colitis 1
• Maintain high vigilance for malignancy: Both cholangiocarcinoma and colorectal cancer risks are substantially elevated 1, 3