.PBC

[Guest guest]

, This group is fine.we also have a group that is just PBCers.I suggest

you belong to both our groups. This one here has some of both PBC/AIH and other

autoimmune liver diseases. The PBC group is much larger.over 2300 members.and

we are a bit over 320. So.this group you tend to have closer friendships of

each other. But, the PBCers will explain more about PBC. I have PBC.. I was

diagnosed way back in 1982, and had a transplant in 2003. I took this LS group

over about 8 years ago.and now we are back as part of the PBC Organization..but

a separate Digest of posts. I myself belong as I said to both and post both

places. As to the inflammation of your bile duct..that is just what it

is.inflamed. Not scary really. In the beginning it seems so much to learn and

can feel overwhelming.but hang in there. You will have lots of people here for

you.ask any questions and we will do our best to answer. I also want to state

one very important thing. It has been shown that people who are diagnosed early

and go on URSO very rarely ever go past stage 2! That is awesome! If they

had URSO back in 1982 I MAY never had needed a transplant. But.I am doing great

now.and of course am still on URSO 250. BTW..AXCAM the makers of URSO 250 and

URSO 500 are our sponsors. ; - ) Joanne C. past owner.now Moderator

www.pbcers.org This is their link to join.as Sandy gave.

What is PBC?

Primary biliary cirrhosis (PBC) is a chronic (long duration) disease

characterized by progressive inflammation and destruction of the small bile

ducts within the liver. What are the bile ducts and what do they do? Lined with

cells named biliary epithelial cells, the bile ducts are tubules that make up a

plumbing system for the liver. The bile ducts along with the gallbladder are

part of what is called the biliary tract.

The plumbing system begins in the liver with very small caliber ducts that

connect to increasingly larger caliber ducts, like a tree in which twigs connect

to small branches that connect to larger branches. In fact, this system is often

referred to as the biliary tree. The large right and left bile ducts, still

within the liver, connect to an even larger common bile duct that runs outside

the liver to the small intestine just beyond the stomach. The common bile duct

connects by the cystic duct to the gallbladder. The gallbladder is a

pear-shaped, expandable, sac-like organ in the biliary system. The branching

bile ducts course through special tissue in the liver, called portal tracts,

which act like conduits for the ducts. In fact, the branching portal tracts

containing the bile ducts also contain the blood vessels that enter and leave

the liver.

The bile ducts carry bile, a fluid that is produced by the liver cells

(hepatocytes) and modified by the biliary lining (epithelial) cells as it flows

through the ducts to the small intestine. Bile contains substances required for

digestion and absorption of fat called bile acids, as well as other compounds

that are waste products, such as the pigment bilirubin. (Bilirubin is a

yellow-orange compound produced by the breakdown of hemoglobin from old red

blood cells.) Bile is stored in the gallbladder between meals and discharged

into the small intestine during digestion of the meals.

The inflammation in PBC starts in the liver's portal tracts and involves the

small bile ducts in these areas. The destruction of the small bile ducts blocks

the normal flow of bile into the gut. The medical term for decreased flow of

bile is cholestasis. (Chole means bile and stasis means failure to flow.)

Cholestasis is a very important aspect of this disease. As the inflammation

continues to destroy more of these bile ducts, it spreads to destroy nearby

liver cells (hepatocytes). As the inflammatory destruction of the hepatocytes

proceeds, scar tissue (fibrosis) forms and spreads through the areas of

destruction.

The combined effects of progressive inflammation, scarring, and toxicity of bile

trapped within hepatocytes (liver cells) culminates in cirrhosis. Cirrhosis is

defined as the stage of disease when there is both widespread scarring of the

liver and clusters (nodules) of hepatocytes reproducing (regenerating)

themselves within the scars. Since cirrhosis occurs only in the later stage of

PBC, the name primary biliary cirrhosis is actually a misnomer for patients in

the earlier stages of the illness. The more technically correct and ponderous

term for PBC, chronic non-suppurative destructive cholangitis, however, has

never been widely used and is unlikely to replace PBC.