Primary sclerosing cholangitis

Bile ducts both inside and outside the liver become inflamed and also scarred, which causes duct blockage. These ducts are important as they are responsible for carrying bile from the liver (Bile works to breakdown fat.) Duct blockage causes a bile buildup which in turn damages the cells of the liver. It can cause liver failure.

The causes of PSC are not known. It is seemingly related in some manner to ulcerative colitis. Theories as to its cause include problems with the immune system, bacteria or even viruses.

It happens more in men than in women. The disease normally starts from age 30 to 60.It can however also start with children. PSC progresses slowly, so the disease can be active for a long time before it is noticedor diagnosed. The Bile duct infections can cause chills and fever. Some major symptoms include tiredness, itching, and jaundice, which causes the eyes and skin to become yellow.

PSC is diagnosed via cholangiography. This is done by X-Ray in conjunction with the injection of a dye into the bile ducts. Cholangiography can be performed as an endoscopic procedure (endoscopic retrograde cholangiopancreatography, ERCP), through radiology or surgery, or with magnetic resonance imaging (MRI) scans. Treatment includes medication to relieve itching, antibiotics to treat infections, and vitamin supplements, as people with PSC are often deficient in vitamins A, D, and K. In some cases, surgery to open major blockages in the common bile duct is also necessary. Liver transplantation (including live transplants whereby a portion of a living donor is given to the donee) is an option if the liver begins to fail.