Defintion
Idiopathic chronic and progressive disorder manifesting as stricturing, inflammation, and fibrosis of intra- and extrahepatic bile ducts.
Aetiology
- Rare, precise cause not determined but increasing evidence of immunological basis.
- Majority have UC or rarely Crohn’s disease.
- Association with number of human leucocyte antigens.
Ulcerative colitis
- 5% of patients with UC develop it
- 75% of patients with PSC are found to have concurrent UC
- Patients with coexisting PSC and UC are at a markedly increased risk for colonic neoplasia (five times)
- Independent on activity of colitis
- An increased risk of development has been demonstrated in patients with human leucocyte antigen (HLA) B8, DR2, DR3 or DR6 haplotype positivity.
- Treatment of PSC with steroids, colectomy or antibiotics is ineffectual.
- Patients undergoing restorative proctocolectomy have a higher subsequent incidence of pouchitis and dysplasia in the ileal pouch mucosa
Presentation
- Early
- Asymptomatic.
- Late
- Jaundice, pruritis, pain, fever and weight loss.
- Abnormal LFTs during investigation of IBD.
- Advanced
- Liver failure.
- Sudden deterioration – may be cholangiocarcinoma (which there is strong association).
Investigation
- LFTs – cholestatic.
- ANCA present in majority, however autoantibodies tested to exclude Primary biliary cirrhosis
- Cholangiography
- Demonstrates diffuse stricturing and attenuated intrahepatic bile ducts.
- ERCP enables brushing and endoscopic therapy.
- MRCP preferred for diagnosis – highly sensitive.
Prognosis
- Poor prognosis (median survival 10 years from diagnosis)
- Development of cholangiocarcinoma or colorectal cancer has become leading cause of death.
Management
- Endoscopic or transhepatic dilatation of short dominant strictures with or without endoscopic stenting has been described as effective
- Liver transplant necessary for end-stage liver disease.
- Recurrence has been reported at 15-25% in transplanted liver
- Indications: persistent jaundice, intractable pruritis, recurrent cholangitis, malnutrition or fatigue. Many undergo before liver failure occurs or when cholangiocarcinoma suspected.
Exclusion of malignant stricture
- Important to exclude cholangiocarcinoma or GB cancer (can occur in 10-36% of PSC)
- Rapid deterioration or dominant stricture should be investigated extensively.
- Serum Ca 19-9 used.
- CT/MRI may show mass lesion in biliary tree.
- PET is superior to differentiate between PSC and cholangiocarcinoma.
- ERCP + brush cytology may give diagnosis;
- ERCP cholangioscopy + targeted biopsies most accurate.
- Laparoscopy – may be used in assessing for transplant.