Hepatology

‘Cirrhosis’ banished from PBC


Experts are urging gastroenterologists to immediately change the name of ‘primary biliary cirrhosis’ to ‘primary biliary cholangitis’, conveniently retaining the same well-established acronym of PBC, to better reflect the modern path of the disease and reduce its stigma.

A multinational group including immunologist Professor Ian Mackay from Monash University, writing in Hepatology, say the change has critical implications for patients.

“It removes the stigmata of cirrhosis and its implications of alcohol abuse,” they say.

“It removes the stigmata of a poor prognosis. Its removal reminds patients that they are living with this syndrome, not dying of it.

“We sincerely call on all medical professionals and all patients and their families and friends worldwide to use from this moment on the name ‘primary biliary cholangitis’ for the disease known by its abbreviation PBC.

“We owe this to our patients and to further our role as caring physicians.”

The group note that the early diagnosis of PBC has dramatically improved with more accurate measurements of markers of cholestasis and better detection of the classic serological hallmark, anti-mitochondrial antibodies.

The prognosis has also dramatically improved, with the introduction of liver transplantation in the 1970s and 1980s and the use of ursodeoxycholic acid therapy from the 1980s and 1990s.

“Today, two out of three patients diagnosed with PBS and treated with UDCA have an expected survival not different from the general population and only a minority will ever develop cirrhosis,” they say.

A series of consensus conferences and surveys in recent years have supported the use of primary biliary cholangitis as the best option to replace the reference to cirrhosis, even though it’s an imperfect description of the disease.

“Alternative proposals like primary small bile duct cholangitis, primary intrahepatic cholangitis, primary small bile duct cholangiopathy, primary biliary/ peripheral (destructive) cholangitis, or primary cholangiohepatitis may come somewhat closer to what we think this inflammatory liver disorder is about,” the group says.

In reality, however, such titles would be too cumbersome for everyday use, both for clinicians and for patients.

The new name for PBC has been adopted by a number of professional societies around the world, and negotiations are underway to incorporate it in ICD-11, the WHO’s disease classification system.

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