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Primary Biliary Cirrhosis, or PBC as it is commonly know, is a rare chronic disorder in which there is destruction of the small bile ducts inside the liver (intrahepatic ducts). It is a condition which almost exclusively affects women over the age of 35 years (less than 10% of cases are men). It seems to progress slowly over many years until the damage becomes so extensive that cirrhosis develops and the process is essentially not reversible. The cause is unknown but it may be due to some underlyinh genetic factor, although only rarely have cases of BPC occurring in more than one member of a family been reported. In its early stages it is not life-threatening.
What are the symptoms of PBC?
Many of the signs and symptoms of PBC are those of chronic liver disease generally, but their nature and the time at which they appear depend on the stage that the disease has reached. Indeed, it is thought that there are probably no symptoms at all for many years and that most patients are diagnosed only after the disease has been present for some time. When symptoms do develop, however, itching (pruritus) is often one of the earliest to appear. This is quite striking and can be extremely severe, even to the extent of making sleep difficult, and it is often not relieved by the usual remedies for itching such as antihistamine drugs or creams. Why this particular form of itching should be associated with this disease is not known. Other symptoms that tend to be more common in PBC than in other chronic liver diseases include dryness of the mouth and eyes, and increased pigmentation of the skin such that it appears unusually tanned. In the later stages, patients gradually become progressively jaundiced as more and more bile ducts are destroyed, which stops bile being excreted properly.
How is PBC diagnosed?
The biochemical liver tests show a so-called cholestatic pattern, with raised blood levels of ALP and GGT (and often also bilirubin) but normal or only slightly raised AST or ALT. In addition, the blood level of one of the immunoglobulins known as IgM is usually also high. The most striking marker of the disease, however, is the presence in the blood of an autoantibody known as anti-mitochondrial antibody (AMA). The finding of AMA together with raised IgM in the blood is virtually diagnostic of PBC. Nonetheless, the doctor will usually wish to do a liver biopsy to determine what stage the disease has reached.
Can PBC be cured?
Unfortunately, at the present time the answer to this question is no. The only "cure" is a liver transplant but the disease may occasionally recur in the new liver. However, a transplant is usually considered necessary only when the disease is quite advenced, which may be 10 or 15 years after it is first diagnosed or not at all (if the disease progresses very slowly).
Several drugs that have been used in the treatment of other autoimmune diseases have been tried extensively over the years but none has proved effective. A number of new drugs are currently being tested and some, such as ursodeoxycholic acid (UDCA), are beginning to show promise but it is early days yet. In general, doctors are able only to treat the symtoms (e.g. itching, dry eyes and dry mouth) and the complications of cirrhosis when this develops.
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