Primary sclerosing cholangitis

Changed by Ayush Goel, 9 Sep 2014

Updates to Article Attributes

Body was changed:

Primary sclerosis cholangitis (PSC) is an uncommon idiopathic inflammatory condition which affects the biliary tree resulting in multiple strictures and eventual cirrhosis.

Epidemiology

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 ( ≈ 4≈4th decade) are most frequently affected .

Clinical presentation

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.

Other associations include:

Pathology

Unfortunately no histological findings are pathognomonic for PSC 2. Frequent findings include 2-4:

  • periductal fibrosis (onionskin lesions)
  • periportal eosinophilic infiltrate
  • paucity of ducts

Radiographic features

The entire biliary tree (both intra and extrahepatic) may be involved, with multiple strictures scattered along its length. In approximately 20% only the intrahepatic and proximal extrahepatic bile ducts are involved ;

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

Ultrasound is able to demonstrate both the changes of cirrhosis and irregularity of bile duct calibre.

CT
  • contour abnormalities and atrophy (see above)
  • marked caudate lobe hypertrophy
  • the atrophied liver is of lower density than the hypertrophied caudate lobe1
  • multiple linear discontinuous low density regions representing dilated intra-hepatic bile duct segments1
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 characterisiccharacteristic 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 ~ 85~85% of cases 9
    • general: ~ 35~35%
    • segmental: ~ 50~50%
  • 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 .

Percutaneous cholangioplasty of dominant strictures is usually performed in a retrograde fashion via ERCP or via a transhepatic approach 6-7 .

Complications

Differential diagnosis

General imaging differential considerations include:

  • -<p><strong>Primary sclerosis cholangitis (PSC)</strong> is an uncommon idiopathic inflammatory condition which affects the biliary tree resulting in multiple <a href="/articles/bile-duct-stricture">strictures</a> and eventual <a href="/articles/cirrhosis">cirrhosis</a>.</p><h4>Epidemiology</h4><p>PSC is strongly associated with <a href="/articles/inflammatory-bowel-disease-ibd">inflammatory bowel disease (IBD)</a> (in 70% cases <sup>6</sup>), especially <a href="/articles/ulcerative-colitis">ulcerative colitis</a> and thus shares similar demographics : young to middle aged males ( ≈ 4<sup>th</sup> decade) are most frequently affected <sup>4 </sup>.</p><h4>Clinical presentation</h4><p>Unlike <a href="/articles/primary-biliary-cirrhosis">primary biliary cirrhosis</a>, antibody titres are usually absent or low <sup>4</sup>. Liver function tests will usually have a cholestatic pattern with elevated alkaline phosphatase (ALP) and bilirubin <sup>2</sup>.</p><p>Other associations include :</p><ul>
  • +<p><strong>Primary sclerosis cholangitis (PSC)</strong> is an uncommon idiopathic inflammatory condition which affects the biliary tree resulting in multiple <a href="/articles/bile-duct-stricture">strictures</a> and eventual <a href="/articles/cirrhosis">cirrhosis</a>.</p><h4>Epidemiology</h4><p>PSC is strongly associated with <a href="/articles/inflammatory-bowel-disease-ibd">inflammatory bowel disease (IBD)</a> (in 70% cases <sup>6</sup>), especially <a href="/articles/ulcerative-colitis">ulcerative colitis</a> and thus shares similar demographics: young to middle aged males (≈4<sup>th</sup> decade) are most frequently affected <sup>4 </sup>.</p><h4>Clinical presentation</h4><p>Unlike <a href="/articles/primary-biliary-cirrhosis">primary biliary cirrhosis</a>, antibody titres are usually absent or low <sup>4</sup>. Liver function tests will usually have a cholestatic pattern with elevated alkaline phosphatase (ALP) and bilirubin <sup>2</sup>.</p><p>Other associations include:</p><ul>
  • -<li><a href="/articles/orbital_pseudotumour">orbital pseudotumour</a></li>
  • -</ul><h4>Pathology</h4><p>Unfortunately no histological findings are pathognomonic for PSC <sup>2</sup>. Frequent findings include <sup>2-4 </sup>:</p><ul>
  • +<li><a href="/articles/orbital-pseudotumour">orbital pseudotumour</a></li>
  • +</ul><h4>Pathology</h4><p>Unfortunately no histological findings are pathognomonic for PSC <sup>2</sup>. Frequent findings include <sup>2-4</sup>:</p><ul>
  • -<li>the atrophied liver is of lower density than the hypertrophied caudate lobe<sup>1</sup>
  • +<li>the atrophied liver is of lower density than the hypertrophied caudate lobe <sup>1</sup>
  • -<li>multiple linear discontinuous low density regions representing dilated intra-hepatic bile duct segments<sup>1</sup>
  • +<li>multiple linear discontinuous low density regions representing dilated intra-hepatic bile duct segments<sup> 1</sup>
  • -</ul><h5>MRCP / Cholangiography / ERCP</h5><p>ERCP remains the gold standard for the depiction of the biliary tree, and also offers the ability to perform cholangioplasty if necessary.</p><p>The characterisic findings on direct imaging of the biliary tree are <sup>2-3,5 </sup>:</p><ul>
  • +</ul><h5>MRCP / Cholangiography / ERCP</h5><p>ERCP remains the gold standard for the depiction of the biliary tree, and also offers the ability to perform cholangioplasty if necessary.</p><p>The characteristic findings on direct imaging of the biliary tree are <sup>2-3,5</sup>:</p><ul>
  • -<li>biliary dilatation - may be present in ~ 85% of cases <sup>9</sup><ul>
  • -<li>general: ~ 35%</li>
  • -<li>segmental: ~ 50%</li>
  • +<li>biliary dilatation: may be present in ~85% of cases <sup>9</sup><ul>
  • +<li>general: ~35%</li>
  • +<li>segmental: ~50%</li>
  • -</ul><h4>Treatment and prognosis</h4><p>The only cure available at present is orthotopic liver transplantation (OLT) with 5 year survival rates approaching 80% <sup>10-11</sup>.  However there is evidence that PSC may recur in 5-20 % of patients post transplantation <sup>10-12</sup>.</p><p>No medical therapy has stood the test of time, with ursodeoxycholic acid, immunosuppression, chelation and antifibrolytic therapy unable to demonstrate consistent benefits over placebo <sup>8 </sup>.</p><p>Percutaneous cholangioplasty of dominant strictures is usually performed in a retrograde fashion via ERCP or via a transhepatic approach <sup>6-7 </sup>.</p><h4>Complications</h4><ul><li>
  • -<a href="/articles/cholangiocarcinoma">cholangiocarcinoma</a> develops in ~ 15% patients <sup>6</sup>
  • -</li></ul><h4>Differential diagnosis</h4><p>General imaging differential considerations include</p><ul>
  • +</ul><h4>Treatment and prognosis</h4><p>The only cure available at present is orthotopic liver transplantation (OLT) with 5 year survival rates approaching 80% <sup>10-11</sup>.  However there is evidence that PSC may recur in 5-20% of patients post transplantation <sup>10-12</sup>.</p><p>No medical therapy has stood the test of time, with ursodeoxycholic acid, immunosuppression, chelation and antifibrolytic therapy unable to demonstrate consistent benefits over placebo <sup>8 </sup>.</p><p>Percutaneous cholangioplasty of dominant strictures is usually performed in a retrograde fashion via ERCP or via a transhepatic approach <sup>6-7 </sup>.</p><h4>Complications</h4><ul><li>
  • +<a href="/articles/cholangiocarcinoma">cholangiocarcinoma</a> develops in ~15% patients <sup>6</sup>
  • +</li></ul><h4>Differential diagnosis</h4><p>General imaging differential considerations include:</p><ul>
  • -<li><a href="/articles/aids_cholangiopathy">AIDS associated cholangitis</a></li>
  • +<li><a href="/articles/aids-cholangiopathy">AIDS associated cholangitis</a></li>
  • -<a href="/articles/hepatic-sarcoidosis">hepatic sarcoidosis</a> - see <a href="/articles/abdominal-manifestations-of-sarcoidosis-1">abdominal manifestations of sarcoidosis</a>
  • +<a href="/articles/hepatic-sarcoidosis">hepatic sarcoidosis</a>: see <a href="/articles/abdominal-manifestations-of-sarcoidosis-1">abdominal manifestations of sarcoidosis</a>
Images Changes:

Image 10 MRI (Thick slab HASTE) ( create )

ADVERTISEMENT: Supporters see fewer/no ads

Updating… Please wait.

 Unable to process the form. Check for errors and try again.

 Thank you for updating your details.