A recent article provides a useful review for primary sclerosing cholangitis (PSC) (Clin Gastroenterol Hepatol 2013; 11: 898-907).
This blog has previously discussed PSC (links below); however, the above reference is succinct and covers the key issues. A couple of points that I found particularly helpful:
- Cholangiocarcinoma (CCA): recommends “consider annual imaging (MRCP or Ultrasound) along with serum CA19-9 levels” to monitor for cholangiocarcinoma. If there is a dominant stricture, proceed with ERCP with brushings. In pediatrics, the age to start screening is less clear, usually not presenting until beyond the late teen years, though CCA has been diagnosed in one case report at 14 years of age (NEJM 2003; 348: 1464).
- Gallbladder cancer (30-40-fold higher risk than general population): If gallbladder polyp identified that is ≥0.8 cm, recommends cholecystectomy. If smaller, may also want to remove if normal synthetic function; otherwise repeat imaging in 3-6 months.
- Colon cancer: colonoscopy every 1-2 years in those with coexistent IBD (70% of patients with PSC have IBD).
Diagnosis: 44-56% of patients are asymptomatic at time of diagnosis, picked up due to abnormal serum liver tests or on cross-sectional imaging.
Small-duct PSC: occurs in the setting of features of PSC (histology, biochemistry) without abnormal cholangiogram. This represents 11-17% of PSC patients and is difficult to identify in patients without IBD. Over time, 25% will develop large-duct PSC. Small-duct PSC does not appear to result in increased risk of CCA.
Overlap syndrome with autoimmune hepatitis: patients with typical PSC but with 5- to 10-fold aminotransferase elevations should be suspected of having an overlap syndrome and may benefit from treatments directed at autoimmune hepatitis. Other features often include histology with an interface hepatitis and the presence of auto-antibodies. This situation is more common in children and young adults.
Immunoglobulin G4-Related sclerosing cholangitis: this occurs most commonly in conjunction with autoimmune pancreatitis. Since steroids can be effective, IgG4 levels should “be tested in all patients with suspected PSC, and, if elevated to consider an evaluation for IgG4-related disease.”
Medical management: “to date, there are no medical therapies that have been proven to alter the natural course of PSC.” The discussion notes that standard doses of ursodeoxycholic acid (UDCA) may have protective effects against colorectal cancer in patients with coexisting IBD. Higher doses of UDCA have been associated with a 2-fold risk of increased disease progression. Specific treatments for dominant strictures, pruritus, metabolic bone disease, and malabsorption are discussed. In patients with cholestasis, monitoring fat-soluble vitamins is important.
Related blog posts:
- Colonic disease and PSC | gutsandgrowth
- Challenges with primary sclerosing cholangitis | gutsandgrowth Numerous references with this blog post.
- Screening for subclinical PSC in IBD? | gutsandgrowth
- Diagnosing autoimmune hepatitis | gutsandgrowth
- Azathioprine metabolite measurement for Autoimmune Hepatitis …