Primary Sclerosing Cholangitis (PSC) is a rare, chronic, progressive inflammatory disease of the intrahepatic (inside the liver) and extrahepatic (outside the liver) bile ducts.
Chronic inflammation of both types causes them to become scarred and thickened, narrowing the path for bile to flow and causing a worsening condition that can lead to cirrhosis and liver failure. About 6 in 100,000 people are diagnosed with PSC.
Many people with early-stage PSC have no symptoms, but are diagnosed after routine blood tests indicate an abnormality in liver function, which is initially detected as elevated levels of liver enzymes.
Patients with early symptoms may feel fatigue and have itching. Patients in whom PSC has progressed may develop jaundice – a yellowing of the skin and eyes. As PSC advances, patients usually develop pain in the upper right abdomen, in addition to the earlier symptoms of fatigue, fever, itching, and jaundice.
The cause of PSC is not clear, but researchers suspect bacteria, viruses, and immune system abnormalities. Men have a much higher incidence of PSC than women: about 70% of PSC patients are men. PSC is associated with ulcerative colitis, an inflammatory bowel disease, and with Crohn's disease. A small percentage of PSC patients have an autoimmune form of the disease which is characterized by rapid onset of symptoms (pain, fever, and jaundice) and disease progression.
NewYork-Presbyterian's digestive disease team includes a number of specialists with extensive experience treating PSC. These physicians work with patients to slow the progression of PSC and preserve liver function for as long as possible. We are also home to a world-renowned NewYork-Presbyterian Transplant Institute, which enables us to treat end-stage PSC with liver transplantation.
If our gastrointestinal specialists suspect PSC, they will perform a number of tests to determine if you have PSC, including:
For early PSC, doctors will prescribe the drug cholestyramine (Questran) to relieve itching, which works by reducing the level of bile acids in a patient's blood and, therefore, the skin. Doctors will also prescribe vitamin and mineral supplements to replace missing nutrients due to malabsorption. Our experts may also prescribe ursodeoxycholic acid, or UDCA, a naturally occurring bile acid to increase the flow of bile and thereby reduce inflammation of bile ducts in early stages of the disease. Side-effects of this drug may include diarrhea, aches, and trouble swallowing.
Our team of specialists monitors every patient with PSC by performing regular examinations and blood tests to determine how the liver and bile ducts are functioning.
If necessary, an ERCP, the same procedure used to diagnosis PSC, can be used to treat it. After visualizing the area, physicians insert a small tube through the endoscope and into the bile ducts to drain them.
When PSC is advanced, a liver transplant may help prolong life. Liver transplants are performed by surgeons in NewYork-Presbyterian's Center for Liver Disease and Transplantation (CLDT).
Patients with advanced PSC are at risk of cirrhosis of the liver and liver failure and consequently will need a liver transplant. Symptoms of cirrhosis include fluid in the abdomen (ascites), swelling of the ankles, frequent infections, internal bleeding from esophageal blood vessel ruptures (varices), mental confusion (encephalopathy), and coma.
PSC frequently causes bacterial infection of the bile ducts, or cholangitis, which occurs as a result of narrowing and scarring of the ducts and the inability of bile to freely flow through them. Symptoms of cholangitis, which is life-threatening and requires immediate medical care, include fever, chills, jaundice, and pain in the upper abdomen. This dangerous complication can lead to a serious infection in the bloodstream, called sepsis, which can damage the lung and kidneys and may result in death.
PSC patients have a higher risk of cholangiocarcinoma, a cancer of the bile ducts. Patients with both PSC and longstanding ulcerative colitis have the highest risk of this type of cancer. (Ulcerative colitis is an inflammatory bowel disease that causes inflammation and sores, called ulcers, in the lining of the rectum and colon, and frequent diarrhea. Ulcers form where inflammation has killed the cells that usually line the colon, then bleed and produce pus.)
PSC may also cause patients to have deficiencies in fat-soluble vitamins. This may lead to: osteoporosis or weak bones caused by vitamin D and calcium deficiencies; bleeding caused by a deficiency in vitamin C; and night blindness caused by a deficiency in vitamin A.
Much of the Department's research has focused on a technique used to treat cholangiocarcinoma, a cancer and complication of PSC. The technique is known as Spyglass and involves placing stents into the bile ducts of the liver. These stents or small tubes enlarge or maintain the size of the ducts and allow bile to flow freely through them.
The other part of SpyGlass is using Photodynamic Therapy. With this, a specific solution is infused into the ducts and then "activated" by exposing it to light of a specific wavelength. The activation kills the targeted cancer cells while leaving the surrounding healthy cells unharmed.