It is an inflammatory strictures with progressive course which affect the biliary tract either intrahepatic or exttrahepatic or both  it is non suppurative cholangitis can lead to secondary biliary cirrhosis and liver failure

  • primary sclerorsing cholangitis
  • Secondary sclerorsing cholangitis

There is no obvious causes may due to autoimmune diseases
Secondary sclerorsing cholangitis
It is associated with other diseases such as ulcerative colitis
Riedel's thyroiditis and retroperitoneal fibrosis
Other causes of biliary strictures
bile duct stones acute cholangitis previous biliary surgeryor toxic agents
Age mean age 35-40 years
Common in male more than female

     It  has an autoimmune reaction  as seen in chronic low­ grade bacterial or viral infection, toxic reaction, and genetic fac­tors have all been suggested to play a role in its pathogenesis

The human leukocyte antigen haplotypes HLA-B8. -DR3 -DQ2, and DRw52Acommonly found in patients with autoimmune diseases  also can be seen in patients with scleros­ing cholangitis  Patients with sclerosing cholan­gitis are at risk for developing cholangiocarcinoma.

Symptoms and signs
  • Some patients may remain asymptomatic for years
  •  Intermittent jaundice
  • fatigue, weight loss
  • pruritus or itching
  •  Abdominal pain
  • Symptoms of acute cholangitis are rare
  •  In several patients with ulcerative colitis, abnormal liver function tests found on routine testing lead to the diagnosis
  • Cyclic  of remissions and exacerbations are typi­cal
  •     Progres lead to secondary biliary cirrhosis and liver failure 
  . The median survival for patients with primary sclerosing cholangitis from the time of diagnosis ranges from 10 to 12 years, and most die from hepatic failure

Liver function test

elevation of alkaline phosphatase and bilirubin may suggest the diagnosis with clinical presentation
 Endoscopic retrograde pancreatocholangiography ERCP

It is the most or only diagnostic methods of sclerorsing cholangitis
   It showing multiple dilatations and strictures (beading) of both the intra- and extrahep­atic biliary tree
 The hepatic duct bifurcation is often the most severely affected segment
 A liver biopsy may not be diag­nostic, but is important to determine the degree of hepatic fibrosis and the presence of cirrhosis
. Sclerosing cholangitis is followed by ERCP and liver biopsies to provide appropriate management


There is no known effective medical therapy for primary scleros­ing cholangitis and no known curative treatment
 Corticosteroids, immunosuppressants, ursodeoxycholic acid, and antibiotics may be given
 Biliary strictures can be dilated and stented ei­ther endoscopically or percutaneously or T tube drainage
Surgical management
  Resection of the extrahepatic bil­iary tree and hepaticojejunostomy has produced reasonable results in patients with extrahepatic and bifurcation strictures but without cirrhosis or significant hepatic fibrosis

Liver transplantation
It is only option in patients with sclerosing cholangitis and advanced liver disease
Recurrent primary sclerosing cholangitis
may require re-transplantation

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