Disorders of the liver, bile duct, or pancreas can cause cholestasis.
The skin and whites of the eyes look yellow, the skin itches, urine is dark, and stools may become light-colored and smell foul.
Laboratory and often imaging tests are needed to identify the cause.
Treatment depends on the cause, but drugs can help relieve itching.
(See also Overview of the Liver and Gallbladder Overview of the Liver and Gallbladder Located in the upper right portion of the abdomen, the liver and gallbladder are interconnected by ducts known as the biliary tract, which drains into the first segment of the small intestine... read more , Gallbladder and Biliary Tract Gallbladder and Biliary Tract The gallbladder is a small, pear-shaped, muscular storage sac that holds bile and is interconnected to the liver by ducts known as the biliary tract. (See also Overview of the Liver and Gallbladder... read more , and Overview of Liver Disease Overview of Liver Disease Liver disease can manifest in many different ways. Characteristic manifestations include Jaundice (a yellowish discoloration of the skin and whites of the eyes) Cholestasis (reduction or stoppage... read more .)
With cholestasis, the flow of bile is impaired at some point between the liver cells (which produce bile) and the duodenum (the first segment of the small intestine). When bile flow is stopped, the pigment bilirubin (a waste product formed when old or damaged red blood cells are broken down) escapes into the bloodstream and accumulates. Normally, bilirubin joins with bile in the liver, moves through the bile ducts into the digestive tract, and is eliminated from the body. Most bilirubin is eliminated in stool, but a small amount is eliminated in urine.
View of the Liver and Gallbladder
Causes of Cholestasis
The causes of cholestasis are divided into two groups: those originating within the liver and those originating outside the liver.
Within the liver
Causes include acute hepatitis Overview of Acute Viral Hepatitis Acute viral hepatitis is inflammation of the liver, generally meaning inflammation caused by infection with one of the five hepatitis viruses. In most people, the inflammation begins suddenly... read more , alcohol-related liver disease Alcohol-Related Liver Disease , 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 with inflammation and scarring of the bile ducts, cirrhosis due to viral hepatitis B or C Overview of Hepatitis Hepatitis is inflammation of the liver. (See also Overview of Acute Viral Hepatitis and Overview of Chronic Hepatitis.) Hepatitis is common throughout the world. Hepatitis can be Acute (short-lived) read more (also with inflammation and scarring of the bile ducts), certain drugs (for example, amoxicillin/clavulanate, chlorpromazine, azathioprine, and oral contraceptives), hormonal effects on bile flow during pregnancy (a condition called cholestasis of pregnancy Cholestasis of pregnancy Some liver disorders occur only during pregnancy. Others (such as gallstones, cirrhosis, or hepatitis) may have been present before the pregnancy, or they may occur coincidentally with the pregnancy... read more ), and cancer that has spread to the liver.
Outside the liver
Causes include a stone in a bile duct 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 , narrowing (stricture) of a bile duct, cancer of a bile duct, cancer of the pancreas, and inflammation of the pancreas (pancreatitis Overview of Pancreatitis Pancreatitis is inflammation of the pancreas. The pancreas is a leaf-shaped organ about 5 inches (about 13 centimeters) long. It is surrounded by the lower edge of the stomach and the first... read more ).
Symptoms of Cholestasis
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 , dark urine, light-colored stools, and generalized itchiness are characteristic symptoms of cholestasis. Jaundice is a yellow color of the skin and eyes that results from excess bilirubin deposited in the skin, and dark urine results from excess bilirubin excreted by the kidneys. The skin itches, possibly because bile products accumulate in the skin. Scratching can damage the skin. Stools may become light-colored because the passage of bilirubin into the intestine is blocked, preventing it from being eliminated from the body in stool. Stools may contain too much fat (a condition called steatorrhea) because bile cannot enter the intestine to help digest fat in foods. Fatty stools may be foul-smelling.
The lack of bile in the intestine also means that calcium and vitamin D are poorly absorbed. If cholestasis persists, a deficiency of these nutrients can cause loss of bone tissue. Vitamin K, which is needed for blood clotting, is also poorly absorbed from the intestine, causing a tendency to bleed easily.
Prolonged jaundice due to cholestasis produces a muddy skin color and fatty yellow deposits in the skin. Whether people have other symptoms, such as abdominal pain, loss of appetite, vomiting, or fever, depends on the cause of cholestasis.
Diagnosis of Cholestasis
If blood test results are abnormal, an imaging test, usually ultrasonography
Sometimes a liver biopsy
A doctor suspects cholestasis in people who have jaundice and tries to determine whether the cause is within or outside the liver on the basis of symptoms and the results of a physical examination.
Recent use of drugs that can cause cholestasis suggests a cause within the liver. Small spiderlike blood vessels visible in the skin (called spider angiomas), an enlarged spleen, and accumulation of fluid within the abdomen (ascites Ascites Ascites is the accumulation of protein-containing (ascitic) fluid within the abdomen. Many disorders can cause ascites, but the most common is high blood pressure in the veins that bring blood... read more )—which are signs of chronic liver disease—also suggest a cause within the liver.
Findings that suggest a cause outside the liver include certain kinds of abdominal pain (such as intermittent pain in the upper right side of the abdomen and sometimes also in the right shoulder) and an enlarged gallbladder (felt during the physical examination or detected by imaging studies).
Some symptoms (such as loss of appetite, nausea, and vomiting) do not indicate whether the cause is within or outside the liver.
Typically, blood tests are done to measure levels of two enzymes (alkaline phosphatase and gamma-glutamyl transpeptidase) that are very high in people with cholestasis. However, if the level of alkaline phosphatase is very high but the level of gamma-glutamyl transpeptidase is normal, the cause of the high level of alkaline phosphatase is probably not cholestasis. A blood test that measures the level of bilirubin indicates the severity of the cholestasis but not its cause.
An imaging study Imaging Tests of the Liver and Gallbladder 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 , usually ultrasonography, is almost always done if blood test results are abnormal. Computed tomography (CT) or sometimes magnetic resonance imaging (MRI) may be done in addition to or instead of ultrasonography. If the cause appears to be within the liver, a liver biopsy Biopsy of the Liver Doctors can obtain a sample of liver tissue during exploratory surgery, but more often they obtain a sample by inserting a hollow needle through the person's skin and into the liver. This type... read more may be done and usually establishes the diagnosis.
If the cause appears to be blockage of the bile ducts, more precise images of these ducts are usually needed. Typically, one of the following is done:
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): A flexible viewing tube (endoscope) is inserted through the mouth and into the small intestine, and a radiopaque contrast agent (which can be seen on x-rays) is injected through the tube into the bile and pancreatic ducts. Then, x-rays are taken.
Magnetic resonance cholangiopancreatography Magnetic Resonance Imaging (MRCP): MRCP is MRI of the bile and pancreatic ducts, with specialized techniques that are used to make the fluid in the ducts appear bright and the surrounding tissues appear dark.
Endoscopic ultrasonography: Images are obtained via an ultrasound probe inserted with a flexible viewing tube (endoscope) through the mouth and into the small intestine.
Treatment of Cholestasis
For bile duct blockages, surgery or endoscopy
For blockages within the liver, various treatments depending on the cause
For itching, cholestyramine
A blockage of the bile ducts can usually be treated with surgery or endoscopy (using a flexible viewing tube with surgical instruments attached).
A blockage within the liver may be treated in various ways depending on the cause. If a drug is the suspected cause, the doctor stops its use. If acute hepatitis is the cause, cholestasis and jaundice usually disappear when hepatitis has run its course. People with cholestasis are advised to avoid or stop using any substance that is toxic to the liver, such as alcohol and certain drugs.
Cholestyramine, taken by mouth, can be used to treat itchiness. This drug binds with certain bile products in the intestine, so they cannot be reabsorbed to irritate the skin.
Unless the liver is severely damaged, taking vitamin K can improve blood clotting.
Supplements of calcium and vitamin D are often taken if the cholestasis persists, but they are not very effective in preventing loss of bone tissue.