Clinical Profile of Primary Biliary Cirrhosis that was Diagnosed as Symptomatic Primary Biliary Cirrhosis according to the Revised Diagnostic Criteria in Japan

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Objective The diagnostic criteria for primary biliary cirrhosis (PBC) in Japan were revised in 2004. The prevalence and prognosis of PBC using the revised criteria have not been reported. This study investigated the prevalence and prognosis of "newly-diagnosed" symptomatic-PBC (ns-PBC), which was defined as asymptomatic PBC (a-PBC) in Japan until 2004. Patients and Methods The clinical features and the prognosis of 207 patients with PBC were retrospectively investigated according to clinical stage. Results The prevalence of ns-PBC was 3.4% and 9.7%, at the time of diagnosis and final evaluation, respectively. The prognosis of ns-PBC was poorer than a-PBC. A total of 7.2% of the patients with a-PBC progressed to ns-PBC during the observation period. These patients had a poorer prognosis than patients who remained asymptomatic. Conclusion Approximately 10% of the PBC patients presented with signs of portal hypertension as an initial symptom. The signs of portal hypertension should therefore be carefully investigated in patients with PBC at the time of diagnosis and during the observation.