Primary sclerosing cholangitis (PSC) is an idiopathic bilious disease. The typical clinical features are right upper abdominal discomfort, fatigue, skin pruritus and weight loss. The histological manifestations are diffuse inflammation of the bile ducts, extensive fibrotic thickening and bile duct strictures. The American Liver Association published a clinical guideline for the diagnosis and management of PSC in the journal Hepatology in February 2010, and its recommendations are partially extracted below. Liu Changjiang, Department of Gastroenterology, General Hospital of Jinan Military Region
I. Recommendations for the diagnosis of PSC
1. Magnetic resonance cholangiography (MRCP) or retrograde cholangiopancreatography (ERCP) is recommended for patients with biochemical tests suggesting stasis of bile (1A).
2. Routine liver biopsy is not recommended for those with typical manifestations of cholangiography (1B).
3. For those with normal MRCP and ERCP examinations, liver biopsy is recommended to clarify small bile duct type PSC (1B).
4. For those with elevated transaminases that cannot be explained by the primary disease, liver biopsy is recommended to diagnose or exclude overlap syndrome (1B).
5. For all possible PSC, serum IgG4 testing is recommended to exclude IgG4-associated sclerosing cholangitis (2C).
II. Recommendations for PSC with significant bile duct stricture
6. In patients with increased bilirubin and/or worsening pruritus, imaging showing progressive bile duct dilatation, and/or cholangitis, immediate ERCP is recommended to rule out significant bile duct stricture (1B).
7. In patients with PSC with significant bile duct stenosis, ERCP may be performed first with or without stenting for biliary dilation (1B).
8. If ERCP is unsuccessful, percutaneous transhepatic cholangiography with or without stenting for biliary dilatation (1B) is recommended.
9. To exclude malignant lesions before treatment, cytobrush or biopsy (1B) is recommended at the time of ERCP.
10. If stenosis cannot be released by the above treatment, surgical treatment (1B) will be performed for some patients with cirrhosis of the liver who have not reached grade C liver function.
11. Along with bile duct dilatation, antibiotic therapy is recommended to effectively control cholangitis (1A).
12. For recurrent bacterial cholangitis, long-term prophylactic antimicrobial application is recommended (1B).
13. For refractory bacterial cholangitis, liver transplantation is recommended for evaluation (1B).
III. Recommendations for concomitant metabolic bone disease
14. To exclude bone deficiency and osteoporosis, bone mineral density examination is performed after the diagnosis of PSC and every 2-3 years thereafter (1B).
15. For hepatic bone deficiency, it is recommended to apply calcium preparation 1.0-1.5 once a day and vitamin D 1000 IU once a day (2C).
16. For hepatic osteoporosis, in addition to the application of calcium preparations and vitamin D treatment, add bisphosphonate treatment (2C).
17. For osteoporosis combined with esophageal varices, intravenous administration of bisphosphonates is recommended (2C).
IV. Recommendations for combined inflammatory bowel disease (IBD)
18. For those with no past symptoms or history of IBD, perform a full colonoscopy when the diagnosis of PSC is confirmed (1A).
19. For PSC combined with IBD, repeat colonoscopy and biopsy every 1-2 years is recommended to exclude malignant changes in the colon (1B).
20. For PSC combined with ulcerative colitis, ursodeoxycholic acid (UDCA) is not recommended to prevent colon cancer (1B).
21. For PSC combined with IBD, it is recommended to follow IBD treatment guidelines for IBD (1B).
V. Recommendations for combined gallbladder disease
22. Review ultrasound annually to rule out gallbladder-occupying lesions (1C).
23. Cholecystectomy should be performed if liver function permits, regardless of the size of the occupancy, in those who present with an occupying gallbladder lesion (1C).
VI. Recommendations for combined bile duct cancer
24. If the patient’s physical and behavioral capacity or liver function indicators deteriorate, the combination of bile duct cancer should be considered (1B).
25. If combined bile duct cancer and no cirrhosis, surgical resection is recommended (2B).
26. If the combination of early bile duct cancer cannot be treated surgically, it is recommended to consider liver transplantation by an experienced transplantation center after adjuvant treatment (1B).
VII. Recommendations on prognostic evaluation
27. For patients with PSC, a prognostic assessment system is not recommended to assess the prognosis because there is no ideal assessment model (1B).
VIII. Recommendations on specific treatment
28. For adult PSC patients, ursodeoxycholic acid therapy is not recommended (1A).
29. For adult PSC patients with combined overlap syndrome, treatment with adrenocorticosteroids or other immunosuppressive agents is recommended (1C).
IX. Recommendations on liver transplantation
30. For patients with advanced cirrhosis, liver transplantation is recommended as a treatment (1A).
31. If bile duct stricture occurs after liver transplantation, other causes of stricture should be excluded before the diagnosis of PSC recurrence (1B).
X. Recommendations for women with PSC pregnancy
32. For women of childbearing age, pregnancy may be allowed under close supervision.
XI. Recommendations for children with PSC
33. In children, the diagnosis of PSC, overlap syndrome and PSC is dependent on liver biopsy (1B).
34. In children with overlap syndrome, immunosuppressive therapy is recommended (1B).
35. In children with PSC, the application of screening and surveillance protocols to follow bile duct cancer is not recommended; however, in children with combined IBD, the development of colon cancer should still be monitored after the diagnosis of PSC is established (1B).
36. For end-stage liver disease due to PSC, liver transplantation is recommended as an effective treatment (1A).
Assessment grading system with recommendations
Strength of recommendation Description
Strong (1) Factors influencing the recommendation include quality of evidence, patient self-reported outcomes, and cost
Weak (2) Inconsistency, low certainty, or high cost of opinion regarding preference and role
Quality of evidence Explanation
High (A) The reliability of the current assessment is unlikely to be changed by future studies
Moderate (B) The reliability of the current assessment is likely to be altered by future studies
Low (C) The reliability of the current assessment is highly likely to be changed by future studies