Primary Biliary Cirrhosis

Primary biliary cirrhosis

        Primary biliary cirrhosis (PBC) is a disease of the liver, just like any other type of cirrhosis. What makes it different and even more dangerous than your typical cirrhosis is that, while it is chronic and progressive just like classical cirrhosis, it also features one other characteristic: it has a cholestastic aspect, meaning that the bile secreted by the liver cannot flow freely from its point of origin to the intestines.

       However, some may argue that all variations of cirrhosis can cause your bile duct to clog, block or simply shut down. That is true. The reason why primary biliary cirrhosis is in a class of its own when it comes to the danger it poses is because of its unknown cause. It has been postulated in recent years that the cause of primary biliary cirrhosis is autoimmune in nature (meaning that your own body is to fault).

       While its cause may be unknown so far, the progress of the disease is certainly not: PBC destroys small - to - medium bile ducts, thus leading to progressive cholestasis (the complete blocking of the bile ducts) and, often, to an end-stage liver disease, usually classical cirrhosis.

       Primary biliary cirrhosis is a somewhat sexist disease: it mainly targets women in the middle to late stages of their lives, normally between the ages of forty and sixty. That does not mean, under any circumstances, that men are not affected by it. Just in smaller numbers.

Primary biliary cirrhosis symptoms

       Unfortunately, while PBC is much more dangerous than regular liver cirrhosis, its symptoms are completely innocuous when trying to differentiate them from other types of liver diseases or even age related physical conditions:

• fatigue: is the primal symptom of this illness. Unfortunately, it is also a major sign of other conditions, like diabetes or heart problems. Also, in women over the age of 45, due to menopause, it is not such an unusual state of being. However, there is a way of telling them apart: primary biliary cirrhosis fatigue is far more severe than the others and just like PBC, it has no certain origin. It can cause physical disability and even psychical instability, all the way to depression and obsessive - compulsive behavior;
• excessive daytime somnolence: usually associated with fatigue, it is a sleep disorder that appears in a large portion of patients suffering from PBC;
• pruritus: is a skin condition that implies the desire to scratch the affected areas. It is present in more than half of the PBC patients, but only a tenth of them suffer from the severe form. Just like the other symptoms above, no cause is known for it.
• physical exams will reveal important clues, as well. The results do depend on the stage of the disease, because in its early phases, they are normal. However, once the illness progresses enough, the findings are more than obvious: hepatomegaly (the enlargement of your liver), hyperpigmentation (you will notice yellow or brown spots all over your body), splenomegaly (the enlargement of your spleene) and, in the final stages, spider nevi (a red dot on your skin, getting redder and bigger with time) and temporal and proximal muscle wasting.

Factors that could cause primary biliary cirrhosis

       Since its causes are still unknown, all that medical doctors can do is offer opinions on what could be the origin of this type of cirrhosis. So far, they have come up with three different categories of factors:

• genetic factors: if your mother, father or siblings have had PBC, you have a more than 50% changes to suffer from that same illness at some point in your lifetime. It has been postulated that an inherited abnormality of immune regulation is to fault;
• infections with organisms belonging to the family Enterobacteriaceae: a significant part of patients that suffer from this type of cirrhosis present infections of their urinary tract with gram - negative bacteria.
• geographical areas: while it is present in the United States of America in generous numbers, it is known to be prevalent in the United Kingdom and the Scandinavian Peninsula. Little to irrelevant numbers of patients with PBC have been reported from Australia, Canada, Estonia and Sweden.

       The treatment of this tricky disease is still in its development stages, though for selected persons, liver transplant has been the only solution. The bad news is that the illness has a high rate of recurrence, despite immunosuppressive therapy.