The diagnosis can be made when there are classical imaging features in the correct clinical context, and secondary causes of cholangitis have been excluded 16.
PSC is strongly associated with inflammatory bowel disease (IBD) (in 70% cases 6), especially ulcerative colitis and thus shares similar demographics: young to middle-aged males (≈4th decade) are most frequently affected 4 .
The average age of diagnosis is 54 years (range 6-93) with increased occurrence in men (63.2%) 15. A large number of asymptomatic individuals are identified upon investigation of persistently deranged liver function tests. Symptomatic individuals commonly present non-specifically with fatigue. More specific symptoms include pruritus, jaundice or GI bleeding 16.
Unfortunately, no histological findings are pathognomonic for PSC 2. Frequent findings include 2-4:
- periductal fibrosis (onion skin lesions)
- periportal eosinophilic infiltrate
- paucity of ducts
- Sjogren syndrome 6
- retroperitoneal fibrosis 6
- mediastinal fibrosis
- Riedel thyroiditis
- orbital pseudotumour
Unlike primary biliary cirrhosis, antibody titres are usually absent or low 4. Liver function tests will usually have a cholestatic pattern with elevated alkaline phosphatase (ALP) and bilirubin 2.
The entire biliary tree (both intra and extrahepatic) may be involved, with multiple strictures scattered along its length. In ~20% only the intrahepatic and proximal extrahepatic bile ducts are involved 4 .
The end result of PSC is cirrhosis which is usually characterised by a markedly distorted biliary tract with atrophy of the entire liver with the exception of the caudate lobe which is hypertrophied in almost all cases (68-98%) 1-5. Atrophy involving the left lobe is a feature which somewhat distinguishes it from cirrhosis from other causes, in which the left lobe is usually hypertrophied 1.
Ultrasound is able to demonstrate both the changes of cirrhosis and irregularity of bile duct calibre. Additionally, brightly echogenic portal triads may be visible.
- contour abnormalities and atrophy (see above)
- marked caudate lobe hypertrophy
- the atrophied liver is of lower density than the hypertrophied caudate lobe 1
- multiple linear discontinuous low-density regions representing dilated intra-hepatic bile duct segments 1
MRCP / cholangiography / ERCP
ERCP remains the gold standard for the depiction of the biliary tree, and also offers the ability to perform cholangioplasty if necessary.
The characteristic findings on direct imaging of the biliary tree are 2-3,5:
- multiple segmental strictures
- typically short segment
- intervening segments are of normal calibre or slightly dilated (beading)
- biliary dilatation: may be present in ~85% of cases 9
- general: ~35%
- segmental: ~50%
- biliary diverticula
- mural irregularities
- distortion of the biliary tree due to associated cirrhosis
Treatment and prognosis
The only cure available at present is orthotopic liver transplantation (OLT) with 5-year survival rates approaching 80% 10-11. However, there is evidence that PSC may recur in 5-20% of patients post-transplantation 10-12.
No medical therapy has stood the test of time, with ursodeoxycholic acid, immunosuppression, chelation and antifibrolytic therapy unable to demonstrate consistent benefits over placebo 8 .
Percutaneous cholangioplasty of dominant strictures is usually performed in a retrograde fashion via ERCP or via a transhepatic approach 6-7 .
- cholangiocarcinoma develops in ~15% patients 6
colorectal cancer 14
- 4x greater risk compared to IBD patients without PSC
- 10x greater risk compared to general population
- hepatocellular carcinoma: appears to be not increased beyond other causes of cirrhosis 14
General imaging differential considerations include:
cirrhosis from other causes
- caudate lobe not as frequently or as markedly enlarged
- left lobe usually also hypertrophied
- secondary sclerosing cholangitis
- cholangiocarcinoma (can also occur as a later complication)
primary biliary cirrhosis
- especially difficult to distinguish when PSC is limited to intrahepatic biliary tree
- young women more frequently affected
- high antibody titres 4
- Alagille syndrome (arteriohepatic dysplasia)
- hepatic sarcoidosis: see abdominal manifestations of sarcoidosis
- 1. Dodd GD, Baron RL, Oliver JH et-al. End-stage primary sclerosing cholangitis: CT findings of hepatic morphology in 36 patients. Radiology. 1999;211 (2): 357-62. Radiology (full text) - Pubmed citation
- 2. Fulcher AS, Turner MA, Franklin KJ et-al. Primary sclerosing cholangitis: evaluation with MR cholangiography-a case-control study. Radiology. 2000;215 (1): 71-80. Radiology (full text) - Pubmed citation
- 3. Ernst O, Asselah T, Sergent G et-al. MR cholangiography in primary sclerosing cholangitis. AJR Am J Roentgenol. 1998;171 (4): 1027-30. AJR Am J Roentgenol (abstract) - Pubmed citation
- 4. Maccarty RL, Larusso NF, Wiesner RH et-al. Primary sclerosing cholangitis: findings on cholangiography and pancreatography. Radiology. 1983;149 (1): 39-44. Radiology (abstract) - Pubmed citation
- 5. Ito K, Mitchell DG, Outwater EK et-al. Primary sclerosing cholangitis: MR imaging features. AJR Am J Roentgenol. 1999;172 (6): 1527-33. AJR Am J Roentgenol (abstract) - Pubmed citation
- 6. Vitellas KM, Keogan MT, Freed KS et-al. Radiologic manifestations of sclerosing cholangitis with emphasis on MR cholangiopancreatography. Radiographics. 20 (4): 959-75. Radiographics (full text) - Pubmed citation
- 7. May GR, Bender CE, Larusso NF et-al. Nonoperative dilatation of dominant strictures in primary sclerosing cholangitis. AJR Am J Roentgenol. 1985;145 (5): 1061-4. AJR Am J Roentgenol (abstract) - Pubmed citation
- 8. Bacon BR, O'Grady JG. Comprehensive clinical hepatology. Mosby Inc. (2006) ISBN:0323036759. Read it at Google Books - Find it at Amazon
- 9. Bader TR, Beavers KL, Semelka RC. MR imaging features of primary sclerosing cholangitis: patterns of cirrhosis in relationship to clinical severity of disease. Radiology. 2003;226 (3): 675-85. doi:10.1148/radiol.2263011623 - Pubmed citation
- 10. Graziadei IW. Recurrence of primary sclerosing cholangitis after liver transplantation. Liver Transpl. 2002;8 (7): 575-81. doi:10.1053/jlts.2002.33952 - Pubmed citation
- 11. Graziadei IW, Wiesner RH, Batts KP et-al. Recurrence of primary sclerosing cholangitis following liver transplantation. Hepatology. 1999;29 (4): 1050-6. doi:10.1002/hep.510290427 - Pubmed citation
- 12. Alexander J, Lord JD, Yeh MM et-al. Risk factors for recurrence of primary sclerosing cholangitis after liver transplantation. Liver Transpl. 2008;14 (2): 245-51. doi:10.1002/lt.21394 - Pubmed citation
- 13. Weber C, Kuhlencordt R, Grotelueschen R et-al. Magnetic resonance cholangiopancreatography in the diagnosis of primary sclerosing cholangitis. Endoscopy. 2008;40 (09): 739-45. doi:10.1055/s-2008-1077509 - Pubmed citation
- 14. Khaderi SA, Sussman NL. Screening for malignancy in primary sclerosing cholangitis (PSC). Curr Gastroenterol Rep. 2015;17 (4): 17. doi:10.1007/s11894-015-0438-0 - Pubmed citation
- 15. Liang H, Manne S, Shick J, Lissoos T, Dolin P. Incidence, prevalence, and natural history of primary sclerosing cholangitis in the United Kingdom. (2017) Medicine. 96 (24): e7116. doi:10.1097/MD.0000000000007116 - Pubmed
- 16. Lindor KD, Kowdley KV, Harrison ME. ACG Clinical Guideline: Primary Sclerosing Cholangitis. (2015) The American journal of gastroenterology. 110 (5): 646-59; quiz 660. doi:10.1038/ajg.2015.112 - Pubmed