Understanding Hydrocephalus: Symptoms, Causes, and Treatments
Hydrocephalus, often referred to as "water on the brain," is a condition where cerebrospinal fluid (CSF) builds up in the cavities (ventricles) deep within the [...]
Medically reviewed by Jerome Albert Ecker | MD, Assistant Professor of Medicine, Duke University - Durham, NC on November 8th, 2023.
Primary sclerosing cholangitis (PSC) is a disease that affects your bile ducts, which are tubes that carry bile from your liver to your small intestine. Bile is a digestive fluid that helps break down fat from food. In people with PSC, the bile ducts become inflamed and develop scar tissue, which can block the flow of bile. This blockage can cause bile to build up in the liver, leading to liver damage and other complications. PSC is more common in males.
PSC often develops slowly, and you may have the disease for years before noticing any symptoms. Some common signs and symptoms of PSC include:
Fatigue
Itchy skin
Yellowing of the skin and eyes (jaundice)
Fever and chills (if bile ducts become infected)
Right upper quadrant abdominal pain
Dark Urine
Pale Stools
The exact cause of PSC is not known, but it may be related to factors such as:
Viral or bacterial infections
Genetic factors
Problems with the immune system
PSC is also linked to inflammatory bowel disease, particularly ulcerative colitis and, less commonly, Crohn's disease. About 75% of people with PSC also have ulcerative colitis, although the connection between these conditions is not fully understood.
To diagnose PSC, your doctor may start by ordering a blood test to check your liver enzyme levels. Abnormal levels of certain enzymes can indicate the presence of PSC. To confirm the diagnosis, your doctor may recommend one of the following imaging tests:
Magnetic resonance cholangiopancreatography (MRCP): This non-invasive test uses magnets and radio waves to create detailed images of your bile ducts.
Endoscopic retrograde cholangiopancreatography (ERCP): During this test, a long, flexible tube called an endoscope is inserted through your mouth and into your digestive tract. Dye is then injected into your bile ducts, and X-rays are taken to visualize any blockages or abnormalities.
Percutaneous transhepatic cholangiography (PTC): This test involves inserting a needle through your skin and into a bile duct in your liver. Dye is injected through the needle, and X-rays are taken to examine your bile ducts.
Currently, there are no medications specifically approved for treating PSC. However, your doctor may recommend the following to help manage symptoms and complications:
Medications to relieve itching
Antibiotics to treat infections in the bile ducts
Vitamin supplements to address any deficiencies
Endoscopic procedures to open blocked bile ducts (during ERCP)
In cases of advanced liver disease or liver failure, a liver transplant may be necessary. Most people with PSC who undergo a liver transplant experience good outcomes and improved quality of life.
If you suspect you may have PSC or are experiencing any of the symptoms mentioned above, it's essential to consult with your healthcare provider for an accurate diagnosis and appropriate treatment plan. Researchers are continually working on developing new therapies for PSC, and your doctor may be able to provide information about clinical trials testing promising medications.
For more information about primary sclerosing cholangitis, visit the following reputable sources:
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