Symptoms begin gradually and include worsening fatigue, itchiness, and, later, jaundice.
An imaging test can confirm the diagnosis.
Treatment focuses on relieving symptoms, but liver transplantation can prolong life.
Bile is a fluid that is produced by the liver and aids in digestion. Bile is transported through small tubes (bile ducts) that carry bile through the liver and then from the liver to the gallbladder and to the small intestine. (See also Overview of Gallbladder and Bile Duct Disorders Overview of Gallbladder and Bile Duct Disorders The liver produces bile, a greenish yellow, thick, sticky fluid. Bile aids digestion by making cholesterol, fats, and fat-soluble vitamins easier to absorb from the intestine. Bile also helps... read more .)
In primary sclerosing cholangitis, bile ducts become inflamed, which can lead to scarring of the bile ducts and liver tissue that progresses, eventually becoming severe (cirrhosis). The scar tissue narrows and blocks the bile ducts. As a result, bile salts, which help the body absorb fats, are not secreted normally. The disorder resembles primary biliary cholangitis Primary Biliary Cholangitis (PBC) Primary biliary cholangitis (PBC) is inflammation with progressive scarring of the bile ducts in the liver. Eventually, the ducts are blocked, the liver becomes scarred, and cirrhosis and liver... read more except that it affects the bile ducts outside the liver as well as those in the liver. The cause is not known but is likely to be autoimmune Autoimmune Disorders An autoimmune disorder is a malfunction of the body's immune system that causes the body to attack its own tissues. What triggers an autoimmune disorder is not known. Symptoms vary depending... read more (when the immune system attacks the body’s own tissues).
Primary sclerosing cholangitis most often affects young men and is diagnosed at an average age of 40. It commonly occurs in people with inflammatory bowel disease Overview of Inflammatory Bowel Disease (IBD) In inflammatory bowel diseases, the intestine (bowel) becomes inflamed, often causing recurring abdominal pain and diarrhea. The two primary types of inflammatory bowel disease (IBD) are Crohn... read more , especially ulcerative colitis Ulcerative Colitis Ulcerative colitis is a chronic inflammatory bowel disease in which the large intestine (colon) becomes inflamed and ulcerated (pitted or eroded), leading to flare-ups (bouts or attacks) of... read more . It tends to occur in families, suggesting that genes may be involved. An infection or injury of the bile ducts may trigger the disorder in people who have genes that make them susceptible to the disorder.
Symptoms of Primary Sclerosing Cholangitis
Symptoms usually begin gradually with worsening fatigue and itchiness. Jaundice Jaundice in Adults In jaundice, the skin and whites of the eyes look yellow. Jaundice occurs when there is too much bilirubin (a yellow pigment) in the blood—a condition called hyperbilirubinemia. (See also Overview... read more (yellowish discoloration of the skin and whites of the eyes) tends to develop later.
Inflammation and recurring infection of the bile ducts (bacterial cholangitis) sometimes occur. Bacterial cholangitis causes attacks of pain in the upper abdomen, jaundice, and fever.
Because bile salts are not secreted normally, people may be unable to absorb enough fats and fat-soluble vitamins (A, D, E, and K). Such impaired bile secretion results in osteoporosis Osteoporosis Osteoporosis is a condition in which a decrease in the density of bones weakens the bones, making breaks (fractures) likely. Aging, estrogen deficiency, low vitamin D or calcium intake, and... read more , easy bruising and bleeding, and stools that are greasy and foul-smelling (steatorrhea). Gallstones Gallstones Gallstones are collections of solid material (predominantly crystals of cholesterol) in the gallbladder. The liver can secrete too much cholesterol, which is carried with bile to the gallbladder... read more and bile duct stones develop in about three fourths of people with primary sclerosing cholangitis. The liver and spleen may enlarge.
As the disorder progresses, symptoms of cirrhosis Symptoms Cirrhosis is the widespread distortion of the liver's internal structure that occurs when a large amount of normal liver tissue is permanently replaced with nonfunctioning scar tissue. The scar... read more develop. Advanced cirrhosis causes the following:
Increased blood pressure in the vein that carries blood from the intestines to the liver (portal hypertension Portal Hypertension Portal hypertension is abnormally high blood pressure in the portal vein (the large vein that brings blood from the intestine to the liver) and its branches. Cirrhosis (scarring that distorts... read more )
Some people have no symptoms until the disorder is advanced and cirrhosis is present. Symptoms may not appear for up to 10 years.
Cancer of the bile ducts (cholangiocarcinoma Tumors of the Bile Ducts and Gallbladder Tumors, both noncancerous and cancerous, within the bile ducts or gallbladder are rare. Ultrasonography or MRI/MRCP can usually detect a tumor in the bile ducts or gallbladder. These cancers... read more ) develops in 10 to 15% of people with primary sclerosing cholangitis.
Usually, primary sclerosing cholangitis worsens gradually. Liver failure typically occurs on average approximately 12 years after the disorder is diagnosed.
Diagnosis of Primary Sclerosing Cholangitis
Liver blood tests
Ultrasonography, followed by other imaging tests
The disorder may be suspected when results of liver tests Liver Blood Tests Liver tests are blood tests that represent a noninvasive way to screen for the presence of liver disease (for example, hepatitis in donated blood) and to measure the severity and progress of... read more , done as part of an annual physical examination or for some unrelated reason, are abnormal. Then, ultrasonography Ultrasonography Imaging tests of the liver, gallbladder, and biliary tract include ultrasonography, radionuclide scanning, computed tomography (CT), magnetic resonance imaging (MRI), endoscopic retrograde cholangiopancreatography... read more is typically done first to check for blockage of bile ducts outside the liver. Tests that can confirm the diagnosis include the following:
Magnetic resonance cholangiopancreatography (MRCP): Magnetic resonance imaging Magnetic Resonance Imaging Imaging tests of the liver, gallbladder, and biliary tract include ultrasonography, radionuclide scanning, computed tomography (CT), magnetic resonance imaging (MRI), endoscopic retrograde cholangiopancreatography... read more (MRI) is used to obtain images of the bile ducts and the pancreatic duct. This test helps confirm primary sclerosing cholangitis and rule out other causes of bile duct obstruction.
Endoscopic retrograde cholangiopancreatography Endoscopic Retrograde Cholangiopancreatography Imaging tests of the liver, gallbladder, and biliary tract include ultrasonography, radionuclide scanning, computed tomography (CT), magnetic resonance imaging (MRI), endoscopic retrograde cholangiopancreatography... read more (ERCP): X-rays are taken after a radiopaque contrast agent, which is visible on x-rays, is injected into the bile ducts through an endoscope (see figure Endoscopic retrograde cholangiopancreatography [ERCP] Understanding Endoscopic Retrograde Cholangiopancreatography ). ERCP is less desirable than MRCP because ERCP is more invasive and requires injection of a contrast agent. However, ERCP can also sometimes be used to treat complications of the disorder.
Blood tests and MRCP may be done regularly to check for cancer of the bile ducts and liver tissue.
When people are diagnosed with primary sclerosing cholangitis, they should also have a colonoscopy with biopsies (see Endoscopy Endoscopy Endoscopy is an examination of internal structures using a flexible viewing tube (endoscope). In addition to examinations, doctors can use endoscopy to do biopsies and give treatment. Endoscopes... read more ) to determine if they also have inflammatory bowel disease Overview of Inflammatory Bowel Disease (IBD) In inflammatory bowel diseases, the intestine (bowel) becomes inflamed, often causing recurring abdominal pain and diarrhea. The two primary types of inflammatory bowel disease (IBD) are Crohn... read more .
Treatment of Primary Sclerosing Cholangitis
Treatment of symptoms and complications
Sometimes liver transplantation
If people have no symptoms, no treatment is required. But at least twice a year, they need to have a physical examination and blood tests to monitor the progression of the disorder.
The drugs ursodeoxycholic acid and cholestyramine may help relieve itching. Recurring bacterial cholangitis is treated with antibiotics. ERCP is done as needed to widen (dilate) the blocked ducts. Sometimes tubes to keep the ducts open (stents) are inserted temporarily.
Liver transplantation Liver Transplantation Liver transplantation is the surgical removal of a healthy liver or sometimes a part of a liver from a living person and then its transfer into a person whose liver no longer functions. (See... read more is the only treatment that prolongs life and can be curative. A liver transplant may be needed in people with cirrhosis Cirrhosis of the Liver Cirrhosis is the widespread distortion of the liver's internal structure that occurs when a large amount of normal liver tissue is permanently replaced with nonfunctioning scar tissue. The scar... read more and related serious complications, in those with recurrent bacterial cholangitis, or for people with bile duct or liver cancer.
If cancer of the bile ducts Tumors of the Bile Ducts and Gallbladder Tumors, both noncancerous and cancerous, within the bile ducts or gallbladder are rare. Ultrasonography or MRI/MRCP can usually detect a tumor in the bile ducts or gallbladder. These cancers... read more develops and surgery to remove the cancer is not possible, stents may be passed through an endoscope and placed in bile ducts that are blocked by the cancer. These stents open the ducts, can improve jaundice, and prevent recurrent infections. If bile duct cancer is limited to the bottom of the liver (where the bile ducts outside of the liver meet the bile ducts inside the liver), a liver transplant Liver Transplantation Liver transplantation is the surgical removal of a healthy liver or sometimes a part of a liver from a living person and then its transfer into a person whose liver no longer functions. (See... read more may be a curative option.
The following are some English-language resources that may be useful. Please note that THE MANUAL is not responsible for the content of these resources.
International Foundation for Functional Gastrointestinal Disorders (IFFGD): A reliable resource that helps people with gastrointestinal disorders manage their health.
National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK): Comprehensive information on how the digestive system works and links to related topics, such as research and treatment options.