Changing nomenclature for PBC from “primary biliary cirrhosis” to “primary biliary cholangitis”

Primary biliary cirrhosis (PBC) is a chronic cholestatic liver disease that frequently develops in middle-aged women.1 In patients with PBC, small bile ducts in the liver are believed to be damaged by autoimmune reactions.2 Patients were diagnosed as having PBC when two or more of the following three features are observed: (i) elevated cholestatic enzymes; (ii) presence of antimitochondrial autoantibodies; and (iii) in particular, histological findings as chronic non-suppurative destructive cholangitis.3 When the disease entity of PBC was established more than 50 years ago,4 advanced liver diseases showing histological findings of cirrhosis were found in most patients with PBC. Consequently, a nomenclature for primary biliary “cirrhosis” was reasonably accepted by all hepatologists and gastroenterologists worldwide. Introduction of antimitochondrial autoantibodies as a tool for screening examination, however, enabled physicians to diagnose patients with chronic cholestatic diseases as having PBC during the earlier stages before developing cirrhosis.5 Moreover, spread of the use of ursodeoxycholic acid as a first-line therapeutic agent contributed to the inhibition of liver disease progression from the non-cirrhotic stage to the cirrhotic stage in a large number of patients with PBC.6 In Japan, approximately 70–80% of patients with PBC were shown to be diagnosed as having “asymptomatic PBC” in recent years.3 Thus, the nomenclature of primary biliary “cirrhosis” is regarded as a misnomer, both from clinical and histological aspects. As a result, patients inevitably misunderstand the stage of their disease and at the same time have to bear the substantial burden in their social activities. During 2014 and 2015, the governing boards of both the European Association for the Study of the Liver (EASL) and the American Association for the Study of Liver Diseases (AASLD) approved the change of nomenclature for PBC from “primary biliary cirrhosis” to “primary biliary cholangitis”. Each published statements in their official journals,7, 8 followed by the publication of identical articles in several major journals in the fields of gastroenterology and hepatology.9-11 In contrast, in Japan, the Intractable Hepato-Biliary Diseases Study Group, supported by Health Labor Science Research Grants from Research on Measures for Intractable Diseases, administered a questionnaire survey regarding the nomenclature for PBC to 18 experts in clinical research of PBC in 2014. Although all agreed to revise the nomenclature for PBC, unanimous consent was not obtained. Of the 18 Japanese experts, 11 supported the nomenclature of “cholangitis”, whereas 5 and 2 experts proposed “cholangiopathy” and other nomenclatures, respectively. “Biliary” and “cholangitis” may appear to be redundant repetition, and “cholangitis” does not exactly reflect the pathological features observed in the liver of patients with PBC. Considering consistency with the nomenclature approved by EASL and AASLD, the Study Group decided to adopt the nomenclature of “primary biliary cholangitis”, to keep a well-known abbreviation “PBC”. However, an alternative nomenclature for PBC, which more accurately represents the disease entity, should be established in the future. In December 2015, the Intractable Hepato-Biliary Diseases Study Group of Japan addressed the proposal regarding revision of the nomenclature for PBC to the governing boards of the Japanese Society of Gastroenterology (JSGE) and the Japan Society of Hepatology (JSH), and the nomenclature “primary biliary cholangitis (PBC)” was approved in January 2015 and March 2015 by JSH and JSGE, respectively. The societies jointly proposed the novel nomenclature to the Japanese Association of Medical Sciences in April 2014, and the nomenclature “primary biliary cholangitis” will be officially used by the Ministry of Health, Labor and Welfare of Japan following discussion by the committee for regulation of medical terms, organized in the Japanese Association of Medical Sciences. We sincerely call on all members of JSGE and JSH to use from this moment on the name “primary biliary cholangitis”, instead of primary biliary “cirrhosis”, for the disease known by its abbreviation PBC.