Primary biliary “cirrhosis”: Time to replace a misnomer
Authors: Inka Wahl, Andreas Feige, Bernd Löwe, Christina Weiler‐Normann, Matthias Rose, Ansgar W. Lohse, Christoph Schramm
Journal: Hepatology (2014)

Potential conflict of interest: Nothing to report. This work was supported by the YAEL Foundation. To the Editor: At present, the vast majority of patients with the diagnosis of primary biliary cirrhosis (PBC), in fact, are diagnosed at an early stage of disease and do not suffer from cirrhosis.1 In addition, approximately two thirds of patients will never reach the cirrhotic stage of disease as a result of early serological diagnosis and widespread treatment with ursodeoxycholic acid. However, once being confronted with this diagnosis term, patients have to deal with the meaning of the word cirrhosis and its connotations both to themselves and their social environment—irrespective of the stage of their liver disease. According to the results of a psychological study we conducted in our hospital, this term suggests deadly consequences to PBC patients and aggravates their perception of being stigmatized as an alcoholic. In our qualitative study, 12 consecutively recruited female PBC patients (mean age: 52 years; range, 21‐70) who had been given the diagnosis recently (5 months, on average; range, 1 day to 2.5 years) were asked, in a semistructured interview, to describe their own initial mental associations and emotional reactions to the diagnostic term PBC, their assumptions of what others' associations might be, and their experiences with actual reactions of their social surrounding to the term. Interview results were evaluated using a qualitative content analysis approach. Shock and anxiety were retrospectively reported as the predominant immediate emotional reactions by patients when receiving the diagnosis PBC. Mental associations, such as "ultimate destination" and "liver collapse," were described as initial thoughts to this diagnosis. One patient stated in the interview: "First, I could not memorize the words 'primary biliary', I just heard 'cirrhosis.' And 'cirrhosis' meant to me, yes, death sentence." Four patients reported that they expect their social environment to consider alcohol consumption as their disease's cause when hearing the diagnosis name. Indeed, two thirds of the patients experienced that their acquaintances associated alcohol with the PBC diagnosis. They had to face comments such as, "Only alcoholics develop liver cirrhosis." Half of the patients described themselves as affected and stigmatized by others' reactions to their diagnosis. Thus, the use of the term cirrhosis in the diagnosis PBC suggests a fatal disease stage to the patients and their social surrounding and promotes their experience as being stigmatized as an alcoholic. In consequence, this term may further reduce the quality of life in these patients. Given the fact that, at present, the majority of PBC patients will never reach a cirrhotic stage of disease, this diagnosis term has become a misnomer, as Rubin et al. called it already in 1965,2 and should be replaced. Thus, we strongly feel that, 50 years after realizing the consequences of this misleading disease term, the time has come for action within the hepatology community and patient support groups to replace this misnomer by an adequate name for the disease.