From the Guidelines
Primary sclerosing cholangitis (PSC) should be diagnosed using magnetic resonance cholangiopancreaticography (MRCP) as the preferred diagnostic test, and treatment should focus on managing symptoms and complications, with liver transplantation being the only definitive treatment for advanced disease. The diagnosis of PSC is made in the presence of typical findings of sclerosing cholangitis on high-quality cholangiography and after exclusion of secondary causes 1. The disease is characterized by inflammation and scarring of the bile ducts, leading to bile duct narrowing and eventual liver damage. PSC is commonly associated with inflammatory bowel disease, particularly ulcerative colitis, and affects adults between 30-50 years old, with a higher prevalence in men.
Some key points to consider in the management of PSC include:
- The use of ursodeoxycholic acid (UDCA) at 13-15 mg/kg/day to improve liver biochemistry, although its effect on disease progression is unclear 1
- Symptom management with cholestyramine for pruritus, antibiotics for cholangitis episodes, and fat-soluble vitamin supplementation
- Endoscopic procedures to dilate strictures and improve bile flow, with the indication for endoscopic intervention ideally discussed in multidisciplinary meetings of hepatologists, biliary endoscopists, and abdominal radiologists 1
- Regular monitoring, including surveillance for cholangiocarcinoma and colorectal cancer, with patients undergoing colonoscopy every 1-2 years and regular liver imaging to monitor for complications 1
It is essential to note that liver transplantation is the only definitive treatment for advanced PSC, and patients with decompensated cirrhosis or hepatocellular carcinoma should be considered for transplantation according to standard guidelines 1. Additionally, patients with recurrent bacterial cholangitis and/or severe pruritus or jaundice despite endoscopic and pharmacological therapy should also be considered for liver transplantation 1.
From the Research
Definition and Characteristics of PSC
- Primary sclerosing cholangitis (PSC) is a chronic biliary inflammation associated with periductular fibrosis of the intrahepatic and extrahepatic bile ducts, leading to strictures, bacterial cholangitis, decompensated liver disease, and need for liver transplantation 2.
- PSC is a rare focal liver disease that affects all races and ages, with a predominance of young males 2.
- There is an up to 88% association with inflammatory bowel disease 2.
Diagnosis and Treatment
- MRI of the biliary tract is the primary imaging technology for diagnosis 2.
- Endoscopic interventions of the bile ducts should be limited to clinically relevant strictures for balloon dilatation, biopsy, and brush cytology 2.
- Ursodeoxycholic acid is widely used since it improves the biochemical parameters of cholestasis and is safe at low doses 2.
- Antibiotics have been used in several case series over the years to treat PSC and have had, in some cases, impressive benefits when compared with other drugs used for treating the disease 3.
Complications and Prognosis
- PSC is associated with malignancies, particularly cholangiocellular carcinoma, and the risk of cholangiocarcinoma is poorly understood 2, 4.
- End-stage liver disease with decompensation is an indication for liver transplantation, with recurrent PSC in up to 38% of patients 2.
- Early diagnosis of PSC-associated cholangiocarcinoma remains difficult, and treatment options are limited, especially when diagnosed at later stages 4.
Current Research and Future Directions
- Several novel therapeutic strategies are in various stages of development, including apical sodium-dependent bile acid transporter and ileal bile acid transporter inhibitors, integrin inhibitors, peroxisome proliferator-activated receptor agonists, CCL24 blockers, recombinant FGF19, CCR2/CCR5 inhibitors, farnesoid X receptor bile acid receptor agonists, and nor-ursodeoxycholic acid 2.
- Manipulation of the gut microbiome, including faecal microbiota transplantation, is also being explored as a potential therapeutic strategy 2.