EXPLAINING OF SCLERORSING CHOLANGITIS

EXPLAINING OF SCLERORSING CHOLANGITIS

Definition

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

Types

• primary sclerorsing cholangitis
• Secondary sclerorsing cholangitis
  

Causes

 primary 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

Incidence

Age mean age 35-40 years

Common in male more than female

  

 Pathology

     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

Investigations

Laboratory

Liver function test

elevation of alkaline phosphatase and bilirubin may suggest the diagnosis with clinical presentation

Radiological

 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

Biopsy

 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

Treatment

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

You can see also

• Gallbladder anatomy
• Biliary duct anatomy including the common bile duct common hepatic duct and its branches and cystic duct anatomy
• Diagnosis or investigations of biliary channels
• Gallbladder function
• Gallbladder stones causes types diagnosis and treatment
• Acute cholecystitis causes types diagnosis and treatment
• Chronic cholecystitis causes types diagnosis and treatment
• Acaclular or non calcular cholecystitis causes diagnosis and treatment
• Ascending cholangitis causes diagnosis and treatment
• Sclerosing cholangitis  types causes diagnosis and treatment
•    Gallbladder cancer causes diagnosis and treatment
• Bile duct cancer or cholangiocarcinoma causes diagnosis and treatment
• Bile duct cysts or choledochal cysts causes types diagnosis and treatment
• Bile ducts injury or strictures causes types diagnosis and treatment
• Problems after gall bladder removal or postcholecystectomy complications

tags:sclerorsing,explaining,cholangitis