I was wondering the difference because I kept hearing conflicting information. I was just told my liver elevation wasn’t acute and I’m getting checked on the 12th for PSC to confirm their suspicion.
As a result, I was told by my primary care doctor that PBC was one of the ‘better’ liver diseases and if it were anything, he’d rather have it. At the same time, I was told that PBC and PSC WERE the same diseases and that PSC was just the technical term. Since I have Ulcerative Colitis, he said that it’s likely that I have it as a result. Yet when I look it up, they are represented as two different diseases and if anything, I’m a little afraid of having PBC since I just took the test even if I’m more likely to have PSC.
Welcome to the community AutoimmunetoStars,
All these different terms can be confusing. I’ll give you a textbook definition of both. They are very similar though. One of our other members may be able to better clarify but PBC starts its damage in the small ducts of the liver eventually damaging the large ducts. vs. PSC affects the larger main ducts first then moving up to the small ducts. But then you add small duct psc vs large duct psc. A lot to comprehend for sure.
I would encourage you to make sure you are seeing a hepatologist tied in with a transplant center to manage your psc care. Stay away from your local GI and primary care in the management of PSC if you are definitely diagnosed with the disease. If MRCP shows PSC, they will want to do an ERCP to be for sure that you have it. The ERCP allows you the doctor to see the insides of your bile ducts visually by sending a scope down into your stomach and up into your bile ducts. It’s going to take some time to fully educate yourself and we are here to help in any way we can. Ok, here are those definitions.
Primary sclerosing cholangitis (PSC) is a chronic, progressive, cholestatic liver disease resulting from inflammation, fibrosis, and destruction of the intrahepatic and extrahepatic bile ducts. This leads to multiple areas of stricturing in the biliary tree and eventually to cirrhosis.
Primary biliary cirrhosis (PBC) is a progressive disease of the liver caused by a buildup of bile within the liver (cholestasis) that results in damage to the small bile ducts that drain bile from the liver. Over time, this pressure build-up destroys the bile ducts leading to liver cell damage.
PBC and PSC are two different conditions (actually, PSC itself is likely many different conditions all manifesting similar symptoms). The underlying cause for either is not known. Both involve chronic inflammation of the bile ducts, eventually resulting in scarring and obstruction of the ducts.
Think of the biliary tree as, well, a tree. The big trunk that connects with the small intestine, pancreas, and gallbladder is outside of the liver and branches into two main sections that enter the liver. These branches continue to branch off into smaller ducts within the liver. PBC inflammation affects the smaller ducts in the liver. PSC inflammation can affect any of the ducts inside or outside of the liver, usually including the really big ones that show up on imaging tests, but can also be limited to just the smaller ducts just like with PBC (this is called small duct PSC). Both PBC and PSC tend to elevate liver function markers of cholestasis (bile duct obstruction) to include alkaline phosphatase (ALP or ALKP) and gamma glutamyl transferase (GGT). Symptoms of cholestasis (itching, jaundice, nausea, weight loss, cholangitis, etc.) can be similar between the two conditions.
PBC affects a greater proportion of women and is usually not accompanied with IBD (UC or Crohn’s). Most people with PBC are positive to the AMA antibody which helps with diagnosis. Imaging tests won’t show any abnormalities. Multiple treatments are available including urso (UDCA) and obeticholic acid (OCA).
PSC affects a greater proportion of men and is usually accompanied with IBD (UC or Crohn’s). There are no conclusive antibodies but those with the more common large-duct form of the disease will show bile ducts with a beaded appearance on imaging tests (usually done with an MRI procedure called an MRCP). If imaging tests are normal and AMA is negative, a liver biopsy can be performed to find abnormalities consistent with small-duct PSC. There are no FDA approved treatments for PSC, although many of us use off-label treatments to include urso and oral vancomycin.