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Primary Sclerosing Cholangitis (PSC)

By

Christina C. Lindenmeyer

, MD, Cleveland Clinic

Last full review/revision Mar 2020| Content last modified Mar 2020
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Primary sclerosing cholangitis (PSC) is patchy inflammation, fibrosis, and strictures of the bile ducts that has no known cause. However, 80% of patients also have inflammatory bowel disease, most often ulcerative colitis. Other associated conditions include connective tissue disorders, autoimmune disorders, and immunodeficiency syndromes, sometimes complicated by opportunistic infections. Fatigue and pruritus develop insidiously and progressively. Diagnosis is by cholangiography (magnetic resonance cholangiopancreatography [MRCP] or endoscopic retrograde cholangiopathy [ERCP]). Liver transplantation is indicated for advanced disease.

PSC is the most common form of sclerosing cholangitis. Most (70%) patients with PSC are men. Mean age at diagnosis is 40 years.

Etiology of PSC

Although the cause is unknown, primary sclerosing cholangitis (PSC) is associated with inflammatory bowel disease Overview of Inflammatory Bowel Disease Inflammatory bowel disease (IBD), which includes Crohn disease and ulcerative colitis, is a relapsing and remitting condition characterized by chronic inflammation at various sites in the gastrointestinal... read more , which is present in 80% of patients. About 5% of patients with ulcerative colitis Ulcerative Colitis Ulcerative colitis is a chronic inflammatory and ulcerative disease arising in the colonic mucosa, characterized most often by bloody diarrhea. Extraintestinal symptoms, particularly arthritis... read more Ulcerative Colitis and about 1% with Crohn disease Crohn Disease Crohn disease is a chronic transmural inflammatory bowel disease that usually affects the distal ileum and colon but may occur in any part of the gastrointestinal tract. Symptoms include diarrhea... read more Crohn Disease develop primary sclerosing cholangitis (PSC). This association and the presence of several autoantibodies (eg, anti-nuclear antibodies [ANA] and perinuclear antineutrophilic antibodies [pANCA]) suggest immune-mediated mechanisms. T cells appear to be involved in the destruction of the bile ducts, implying disordered cellular immunity. A genetic predisposition is suggested by a tendency for the disorder to develop in multiple family members and a higher frequency in people with HLAB8 and HLADR3, which are often correlated with autoimmune disorders. An unknown trigger (eg, bacterial infection, ischemic duct injury) probably causes PSC to develop in genetically predisposed people.

Symptoms and Signs of PSC

The presence of both PSC and inflammatory bowel disease increases the risk of colorectal carcinoma, regardless of whether a liver transplantation Liver Transplantation Liver transplantation is the 2nd most common type of solid organ transplantation. (See also Overview of Transplantation.) Indications for liver transplantation include Cirrhosis (70% of transplantations... read more has been done for PSC. Cholangiocarcinoma develops in 10 to 15% of patients.

Diagnosis of PSC

  • Magnetic resonance cholangiopancreatography (MRCP)

Imaging of the hepatobiliary system begins with ultrasonography to exclude extrahepatic biliary obstruction. Although ultrasonography or CT can show ductal dilation, diagnosis requires cholangiography to show multiple strictures and dilations in the intrahepatic and extrahepatic bile ducts. Cholangiography should begin with magnetic resonance cholangiopancreatography Imaging is essential for accurately diagnosing biliary tract disorders and is important for detecting focal liver lesions (eg, abscess, tumor). It is limited in detecting and diagnosing diffuse... read more (MRCP). Endoscopic retrograde cholangiopathy Endoscopic retrograde cholangiopancreatography (ERCP) Imaging is essential for accurately diagnosing biliary tract disorders and is important for detecting focal liver lesions (eg, abscess, tumor). It is limited in detecting and diagnosing diffuse... read more Endoscopic retrograde cholangiopancreatography (ERCP) (ERCP) is usually a 2nd choice because it is invasive. Liver biopsy Liver Biopsy Liver biopsy provides histologic information about liver structure and evidence of liver injury (type and degree, any fibrosis); this information can be essential not only to diagnosis but also... read more is usually not required for diagnosis; when done, it shows bile duct proliferation, periductal fibrosis, inflammation, and loss of bile ducts. With disease progression, periductal fibrosis extends from the portal regions and eventually leads to secondary biliary cirrhosis.

Diagnosis reference

  • Bowlus CL, Lim JK, Lindor KD: AGA Clinical practice update on surveillance for hepatobiliary cancers in patients with primary sclerosing cholangitis: Expert review. Clin Gastroenterol Hepatol 17(12):2416-2422, 2019. doi: 10.1016/j.cgh.2019.07.011.

Treatment of PSC

  • Supportive care

  • Endoscopic retrograde cholangiopancreatography (ERCP) dilation for major (dominant) strictures

  • Transplantation for recurrent bacterial cholangitis or complications of liver failure

Asymptomatic patients usually require only monitoring (eg, physical examination and liver tests twice/yr) and, if adults, periodic imaging and measurement of CA 19-9 for gallbladder cancer and cholangiocarcinoma screening. Ursodeoxycholic acid (up to 20 mg/kg/day) reduces itching and improves biochemical markers but not survival. Chronic cholestasis and cirrhosis require supportive treatment. Episodes of bacterial cholangitis warrant antibiotics and therapeutic ERCP Endoscopic retrograde cholangiopancreatography (ERCP) Imaging is essential for accurately diagnosing biliary tract disorders and is important for detecting focal liver lesions (eg, abscess, tumor). It is limited in detecting and diagnosing diffuse... read more Endoscopic retrograde cholangiopancreatography (ERCP) as needed (1) Treatment reference Primary sclerosing cholangitis (PSC) is patchy inflammation, fibrosis, and strictures of the bile ducts that has no known cause. However, 80% of patients also have inflammatory bowel disease... read more . If a single stricture appears to be the major cause of obstruction (a dominant stricture, found in about 20% of patients), ERCP dilation (with brush cytology and fluorescence in situ hybridization [FISH] to screen for cholangiocarcinoma) and stenting can relieve symptoms.

Treatment reference

  • Aabakken L, Karlsen TH, Albert J, et al: Role of endoscopy in primary sclerosing cholangitis: European Society of Gastrointestinal Endoscopy (ESGE) and European Association for the Study of the Liver (EASL) Clinical Guideline. Endoscopy 49(6):588-608, 2017. doi: 10.1055/s-0043-107029.

Key Points

  • Most (80%) patients with PSC have inflammatory bowel disease, usually ulcerative colitis, and many have autoantibodies.

  • Suspect PSC if patients, particularly those with inflammatory bowel disease, have an unexplained cholestatic pattern of abnormalities in liver function tests.

  • Exclude extrahepatic biliary obstruction by ultrasonography, then do MRCP (or, as a second choice, ERCP).

  • Monitor patients with periodic liver testing, screen regularly for gallbladder cancer and cholangiocarcinoma, and treat symptoms and complications (eg, ERCP to evaluate and treat dominant strictures).

  • Consider liver transplantation if recurrent cholangitis or complications of liver failure develop.

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