Primary Sclerosing Cholangitis
What is primary sclerosing cholangitis (PSC)?
Primary sclerosing cholangitis (PSC) is a chronic disorder of the liver in which the bile ducts outside the liver (the extrahepatic bile ducts) and often the bile ducts inside the liver (the intrahepatic bile ducts) become inflamed, thickened (sclerotic), narrowed, and finally obstructed. This is a progressive process that can in time destroy the bile ducts.
Primary sclerosing cholangitis is also commonly called idiopathic sclerosing cholangitis ("idiopathic" means the cause is unknown) or just sclerosing cholangitis.
What causes primary sclerosing cholangitis?
The cause of sclerosing cholangitis is not known.
Does primary sclerosing cholangitis occur in association with other diseases?
PSC can occur in isolated form (by itself) or in association with other diseases, including: - Inflammatory bowel disease, especially with ulcerative colitis;
- Certain uncommon diseases such as multifocal fibrosclerosis syndrome, Riedel's struma, and pseudotumor of the orbit; and
- AIDS.
In AIDS, of course, the changes in the biliary tract are not idiopathic (of unknown origin), but are due to infection. The infectious agents include mycoplasma, cytomegalovirus, and others. Changes in the biliary tract are quite common in AIDS and are similar to those seen in PSC.
What are the symptoms of primary sclerosing cholangitis?
PSC often triggers jaundice (yellowing), generalized pruritus (itching all over the body), upper abdominal pain, and infection.
As PSC progresses, the disease causes cirrhosis of the liver (irreversible scarring of the liver) and liver failure, leading to the consideration of liver transplantation. PSC is, in fact, one of the more common reasons for a liver transplant.
How is the diagnosis of primary sclerosing cholangitis made?
The diagnosis of PSC is suspected from the symptoms and signs and abnormal laboratory tests and then is confirmed by demonstration of thickened bile ducts using a special radiological test called cholangiography in which dye is injected into the bile ducts and then x-rays are taken of the injected ducts.
How is primary sclerosing cholangitis treated?
The treatment of PSC (short of liver transplantation) includes the drug cholestyramine (Questran) to diminish itching, antibiotics for infection, and vitamin D and calcium to prevent bone loss (osteoporosis). Sometimes, balloon dilatation (a procedure in which the bile ducts are stretched open) or surgery to bypass an obstructed bile duct are performed.
What is the prognosis (long-term outlook) for patients with primary sclerosing cholangitis?
The precise prognosis depends on the age of the person, their degree of jaundice (based on the bilirubin level), how advanced the PSC is by liver biopsy (whether cirrhosis has developed or not), and the size of the spleen (splenomegaly). The expected duration of survival can be more accurately predicted based on these factors.
The long-term prognosis for PSC is poor. Most patients die within 10 years of the time of diagnosis without liver transplantation. Transplantation can be a lifesaver for patients with this disease.
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