A disease of the liver’s bile ducts, this condition’s cause is currently unknown. However, it is linked to inflammatory bowel disease, or IBD.
The Condition
This disease has scar formation in the bile ducts due to inflammation. It will also narrow the bile ducts over time. There will be damage to the liver and the liver cells due to this back up of bile. Long term, it can lead to liver failure and cirrhosis of the liver.
Symptoms
The common symptoms are fatigue and itching with jaundice of the skin and eyes. If there is an infection present there can be fever and chills as well. A person can have the condition and not have signs for many years. It can lead to vitamin deficiencies as the condition progresses.
Diagnosis
There will be many tests ran. One is a blood test where there is a check on the liver enzymes. Then a cholangiography will show the bile ducts on imaging. That is done either by an endoscopic retrograde cholangiopancreatography, or ERCP, by a percutaneous transhepathic cholangiography, or by amagnetic resonance cholangiopancreatography, or MRCP. There can also be a liver biopsy done or an ultrasound to see the liver.
Treatment
At the moment there is no treatment that slows the progression of the condition or that cures the disease. There is only management of symptoms and their complications. There can be antibiotics for the infection, reopening blocked bile ducts, different medications for the itching. If the liver starts to fail there is always the option of liver transplantation. Vitamins can help with the nutritional deficiencies if there are signs of that.
Source: National Digestive Diseases Information Clearinghouse