Hepatobiliary: Primary Sclerosing Cholangitis (PSC)

Primary sclerosing cholangitis is a chronic disease that scars the bile ducts inside and outside the liver. It is strongly linked with ulcerative colitis. PSC raises the risk of cancers in the bile ducts and gallbladder, and it changes how colon cancer surveillance is done in ulcerative colitis. Early recognition and structured liver monitoring help prevent complications. (pubmed.ncbi.nlm.nih.gov)

Key takeaways

• PSC is closely tied to IBD, especially ulcerative colitis. (pubmed.ncbi.nlm.nih.gov)

• Anyone with PSC and IBD needs annual colonoscopy from the time PSC is diagnosed. (academic.oup.com)

• Yearly MRI/MRCP, with or without CA 19-9 blood test, is advised to watch for bile duct and gallbladder cancers. (guidelinecentral.com)

• Gallbladder polyps larger than 8 mm usually warrant surgery; smaller ones are imaged every 6 months. (guidelinecentral.com)

• Ursodeoxycholic acid may be considered at standard doses in selected adults, but high doses are harmful. (journals.lww.com)

What PSC is and why it matters in IBD

Primary sclerosing cholangitis (PSC) causes ongoing inflammation and scarring of the bile ducts. Over time, scarring can block bile flow, leading to cholestasis, cirrhosis, and liver failure. Most people with PSC also have inflammatory bowel disease, more often ulcerative colitis than Crohn’s disease. Pooled studies suggest about 2 to 3 of every 100 people with ulcerative colitis have PSC, while about 7 in 10 people with PSC have IBD. (pubmed.ncbi.nlm.nih.gov)

Who gets PSC with IBD

PSC-IBD often shows a distinct pattern in the colon. Extensive colitis is common, symptoms may be mild, and colorectal neoplasia tends to occur more in the right colon. This higher colon cancer risk drives earlier and more frequent surveillance. (pmc.ncbi.nlm.nih.gov)

How PSC is diagnosed

Persistent elevation of cholestatic liver tests, especially alkaline phosphatase, should prompt evaluation. Magnetic resonance cholangiopancreatography (MRCP) is the preferred imaging test to show typical multifocal bile duct narrowings and dilations. Endoscopic retrograde cholangiopancreatography (ERCP) is reserved for treatment or when sampling is needed, not for initial diagnosis. Liver biopsy is mainly used when small‑duct PSC or autoimmune hepatitis overlap is suspected. (academic.oup.com)

Cancer risks and colon surveillance in PSC‑IBD

PSC raises the risk of cholangiocarcinoma and gallbladder cancer. Annual imaging, preferably MRI/MRCP, with or without CA 19‑9, is advised for adults. Routine cancer surveillance is not recommended for small‑duct PSC or for those under 18. Gallbladder polyps greater than 8 mm generally need cholecystectomy; smaller polyps are followed every 6 months by ultrasound. (guidelinecentral.com)

PSC also changes colon cancer surveillance. For anyone with IBD and PSC, colonoscopy with high‑quality inspection should start at PSC diagnosis and repeat every year, even if colitis seems quiet. Dye‑spray chromoendoscopy or virtual chromoendoscopy improves detection when available. (academic.oup.com)

A practical liver monitoring plan

• At ulcerative colitis diagnosis and at routine follow‑up, check liver tests. Repeated cholestatic abnormalities should trigger PSC workup with MRCP. (academic.oup.com)

• For confirmed PSC, review labs every 6 to 12 months, and consider noninvasive fibrosis assessment over time. Assess bone health with a baseline DEXA scan. (reference.medscape.com)

• Vaccinate against hepatitis A and hepatitis B if not immune, since any chronic liver disease raises risk from these infections. (cdc.gov)

Managing symptoms and dominant strictures

Itching from cholestasis can be managed stepwise: start with cholestyramine, then consider rifampin, naltrexone, or sertraline if needed. Monitor for drug interactions and liver toxicity, especially with rifampin. Refractory cases may need specialist procedures. (pmc.ncbi.nlm.nih.gov)

If a tight bile duct narrowing causes jaundice, cholangitis, or severe itch, ERCP with careful balloon dilation is preferred. Routine stenting is avoided because it raises complication risks; very short‑term stents are used only when necessary. Give antibiotics around ERCP to reduce cholangitis risk. Include cytology and fluorescence in situ hybridization (FISH) sampling if cancer is a concern. (pubmed.ncbi.nlm.nih.gov)

Medicines: what is known and unknown

No medicine has proven to stop PSC progression. Ursodeoxycholic acid (UDCA) can be considered at 13 to 23 mg/kg/day in adults with persistently abnormal cholestatic labs, and continued only if there is a meaningful lab or symptom response after about 12 months. High‑dose UDCA, about 28 to 30 mg/kg/day, should be avoided because it worsens outcomes. Oral vancomycin is not recommended outside trials. (journals.lww.com)

Team‑based care and transplant planning

Everyone with PSC‑IBD should have joint care with a hepatologist experienced in PSC. Early referral to transplant centers helps plan for complications, even years before transplant is needed. About half of patients may eventually need a liver transplant. After transplant, close IBD and cancer surveillance continues. (academic.oup.com)

Surveillance highlights in PSC‑IBD

Red flags that need urgent care

• Fever with chills, right‑upper‑belly pain, or jaundice, which can signal bacterial cholangitis.

• Worsening jaundice, severe itching, weight loss, or dark urine and pale stools.

• New or rapidly growing gallbladder polyp. (pubmed.ncbi.nlm.nih.gov)

FAQs

How often should patients with ulcerative colitis, but without PSC, have liver tests

Liver tests are commonly checked at baseline and at routine IBD visits. Ongoing cholestatic elevations should prompt evaluation for PSC with MRCP. Frequency may increase when using medicines that affect the liver. (academic.oup.com)

Does colectomy remove the need for colon surveillance in PSC

No. Even after colectomy with a pouch, many experts advise continued surveillance, often annually in higher‑risk groups such as PSC. Plans are individualized by the care team. (e-guide.ecco-ibd.eu)

Is PSC more common in men

Yes. PSC occurs more often in men, and most patients with PSC have coexisting IBD, usually ulcerative colitis. (pubmed.ncbi.nlm.nih.gov)