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Clarity, Progress can be Found in Nomenclature
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As we learn more about a disease, we not only have the opportunity to develop new therapeutics, but also new diagnostics and better characterization.
This is true especially for PBC, which was first termed primary biliary cirrhosis. Now that we recognize that most people with PBC are found so early, stigmatizing someone with a word like cirrhosis can be very traumatic and misleading because patients already have difficulty understanding fibrosis progression. Further, there’s a lot of societal stigmatization with cirrhosis; most people think you must have drank alcohol excessively or done something bad to get cirrhosis.
It is a disadvantage to characterize everyone with an immune disease as cirrhotic regardless of stage. We also recognize that this is a disease that now, with early intervention, life expectancy can be the same as the general population. It’s really important to call a spade a spade.
Primary biliary cholangitis is an immune disease that targets the small bile ducts and does not always progress to cirrhosis.
This is especially important because we now have a salvage therapeutic to offer to people who do not fully respond to prior standard of care, ursodeoxycholic acid. The horizon of drug development holds many different agents that will be efficacious in more than one disease state, and PBC is one of those.
Some of the NASH therapies, for example, may also have roles in other diseases like PBC and there are other FXR agonists that will have a role against PBC. Improved characterization and understanding that this disease can be controlled rather than something that is found at an advanced stage is critical to our moving forward.