In 2015, the disease originally known as “primary biliary cirrhosis” became “primary biliary cholangitis” (PBC). According to experts, the new name better reflects true patient experiences.
Although the change from “cirrhosis” to “cholangitis” may seem small, it represents decades of improved understanding of the progression of the disease.
Cirrhosis versus cholangitis
Cirrhosis occurs when the liver becomes permanently scarred, replacing the healthy liver tissue with scar tissue. Over time this leads to liver failure, as scar tissue interferes with normal liver function and blocks blood flow throughout the organ.
Read more about PBC prognosis
Cholangitis, on the other hand, refers to inflammation of the bile ducts of the liver. The bile ducts are responsible for transporting bile into the gallbladder and small intestine, where it plays a role in digestion and removing waste.
Many of the symptoms of cirrhosis and cholangitis may overlap, including:
- Fatigue.
- Itching.
- Weight loss.
- Weakness.
- Jaundice.
Today, PBC is classified into four stages, the last of which is cirrhosis. Progression to cirrhosis isn’t guaranteed in people with PBC; even among those who do develop cirrhosis, the timing of progression might vary widely from person to person.
Additionally, cholangitis isn’t the only pathway to cirrhosis. Other causes include fatty liver, alcohol consumption and hepatitis B or C infection.
How did PBC become ‘primary biliary cholangitis’?
Although PBC has been acknowledged by medical professionals since the 1850s, the disease officially received the name “primary biliary cirrhosis” in 1950. At the time, many felt that this term described the disease process fairly accurately, since most patients were already experiencing cirrhosis by the time they were diagnosed.
In 1959, Dame Sheila Sherlock began advocating against the term “cirrhosis,” since many of her patients with PBC didn’t show signs of cirrhosis when they were first diagnosed.
In 2014, patient advocates called for a change in disease name at the European Association for the Study of the Liver Monothematic Conference on Primary Biliary Cirrhosis. They stated that the current name was not only medically inaccurate, but also created stigma and misunderstanding, as to those unfamiliar with the condition the term “cirrhosis” could imply it was related to alcohol abuse.
After many discussions, medical professionals around the world agreed that the name “primary biliary cholangitis” was a much more accurate term that, conveniently, had the same abbreviation as “primary biliary cirrhosis.”
Is ‘primary biliary cholangitis’ the best term for this disease?
Some scientists believe that the name “primary biliary cholangitis” still isn’t the best name for this disease. Critics argue that the new name may cause individuals to confuse PBC with other types of immune cholangitis. Others feel that the name isn’t specific enough and reflects a vague understanding of the disease process.
Though some may feel that “primary biliary cholangitis” isn’t the perfect description of the condition, most agree that the name is a significant improvement from “primary biliary cirrhosis.”
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