Primary sclerosing cholangitis is a chronic disease of the bile ducts that channel bile from the liver into the intestines. Intrahepatic and extrahepatic bile ducts become inflamed, scarred and thickened (sclerotic), narrowed and, lastly, obstructed. The disease progresses with time.
Obstructions in the bile ducts usher in infection. Bile duct infections, called cholangitis, can cause scarring in the liver and, eventually, the development of cirrhosis or liver failure. If primary sclerosing cholangitis progresses to one of these serious conditions, a liver transplant may be necessary for survival.
The cause of primary sclerosing cholangitis is unknown. Some experts believe that an underlying autoimmune disorder is the cause in at least a small number of individuals diagnosed with PSC. It frequently occurs in patients with inflammatory bowel disease.
In early PSC there are commonly no symptoms.
Nonspecific early symptoms of PSC are:
As the condition progresses symptoms become more likely, including:
Late stage PSC is likely to cause:
In individuals with an underlying autoimmune condition, symptoms may be present in early stages of the disease.
Radiological testing can confirm a PSC diagnosis and rule out other conditions which cause similar symptoms, such as primary biliary cirrhosis and gallstones.
One indication of PSC is elevated alkaline phosphates, which can be detected with a blood test.
Blood tests may also be used to check liver enzymes AST and ALT, as well as AMA (antimitochondrial antibody elevation).
Sometimes an invasive endoscopic retrograde cholangio-pancreatograph (ERCP) or the noninvasive magnetic resonance cholangio-pancreatograph are used for intrahepatic or extrahepatic duct visualization. In the presence of PSC the ducts will appear very narrow in many areas with widening sections in between. Invasive techniques are more risky, presenting a 5-6% chance of causing acute pancreatitis.
Individuals with PSC are at a higher risk of developing ulcerative colitis. As a result, PSC patients are also at risk for developing colon cancer. A colonoscopy is important to diagnose ulcerative colitis and precancerous or early cancerous conditions.
Medications such as antihistamines and cholestyramine are given to target specific symptoms of PSC, such as itchy skin (pruritus). Antibiotics are given for infections. Calcium and vitamin D supplements are sometimes given to prevent bone loss and osteoporosis.
Other treatments for PSC can include
ERCP with balloon dilation and/or bile duct stents to stretch the bile ducts.
If individuals with PSC and advanced cirrhosis, a liver transplant may be needed.
Primary sclerosing cholangitis is rare, affecting about 6 in 100,000 people. More men than women have PSC, with 70% of cases occurring in men. The average age for diagnosis of primary sclerosing cholangitis is 40. Primary sclerosing cholangitis often occurs co-morbidly with ulcerative colitis or Crohn's disease.