Long-term prognosis of primary biliary cirrhosis (PBC) in Japan and analysis of the factors of stage progression in asymptomatic PBC (a-PBC)

Nakano, Toshiaki; Inoue, Katsumi; Hirohara, Junko; Arita, Seizaburo; Higuchi, Kenichi; Omata, Masao; Toda, Gotaro

doi:10.1016/s1386-6346(01)00148-6

Cited by 47 publications

(43 citation statements)

References 13 publications

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“…The survival rate of patients in stage I and II was similar to that in the control population, whereas in treated patients ascertained at late histologic stages, the probability of death or liver transplantation remained significantly increased (23). A report from Japan shows that UDCA administration was a useful prognostic indicator among asymptomatic patients (20). These findings have important implications for future UDCA treatment strategies from asymptomatic stage in PBC.…”

Section: Alteration Of Natural History By Ursodeoxycholic Acidmentioning

confidence: 61%

Risk Factors and Prediction of Long-term Outcome in Primary Biliary Cirrhosis

et al. 2011

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The natural history of the disease varies greatly among individual patients with primary biliary cirrhosis (PBC). Some patients live long without any symptoms while other patients present jaundice and develop hepatic failure in early phases of the disease. Previous studies showed that the natural course of PBC is altered by the use of ursodeoxy cholic acid (UDCA). In this review we discuss variation in the natural course of the disease and it's alteration by UDCA, and risk factors that predict disease progression. Based on clinical observations, there are three types of clinical evolution in PBC: 1) minimal to slow progression over several years; 2) rapid progression to jaundice and hepatic failure, and 3) progression to portal hypertension without developing deep jaundice. Notably, based on our analyses accelerated progression to jaundice and liver failure are reflected by a sustained serologic presence of anti-gp210 antibodies whereas patients with portal hypertension in the absence of jaundice have anti-centromere autoantibodies. These observations highlight the clinical importance of antinuclear antibody analysis in patients with PBC.

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Section: Alteration Of Natural History By Ursodeoxycholic Acidmentioning

confidence: 61%

Risk Factors and Prediction of Long-term Outcome in Primary Biliary Cirrhosis

et al. 2011

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“…8 Patients in the present study had lower frequencies of complicated autoimmune diseases than those in western countries. Other studies in Japan have also reported lower complications of these diseases, 5,6,7 but the difference in frequency of complicated autoimmune diseases between Japanese and Western people have not been clearly explained. Polymorphism of the human leukocyte antigen (HLA) might be related to the difference in races.…”

Section: Complicated Autoimmune Diseasesmentioning

confidence: 83%

A Cross-Sectional Study of Primary Biliary Cirrhosis in Japan: Utilization of Clinical Data When Patients Applied to Receive Public Financial Aid

Sakauchi

Mori

Zeniya

et al. 2005

Journal of Epidemiology

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BACKGROUND: There have not been many reports regarding primary biliary cirrhosis (PBC) in Asia. We conducted a cross-sectional study of PBC in Japan. METHODS: In fiscal year 1999, 9,761 patients with symptomatic PBC were registered to receive public financial aid from the Ministry of Health, Labour and Welfare of Japan. For our cross-sectional study we chose 5,805 patients whose clinical data had been written between 1999 and 2000, and statistically analyzed the data, including sex, age, major symptoms, and laboratory data. RESULTS: Our study estimated that the male-to-female ratio was 1:8.0. The median ages of male and female patients were 59 and 60 years, respectively. The major symptoms and physical findings were as follows: pruritus 53.3%, jaundice 11.3%, xanthomas 5.8%, splenomegaly 38.1%, and esophageal varices 19.1%. Antimitochondrial antibody (AMA) was positive in 86.6%, but its positive rate was lower among Japanese patients than among those in western countries. IgM levels were higher among AMA-positive patients than among AMA-negative patients. Regarding Sjögren’s syndrome, rheumatoid arthritis, chronic thyroiditis, and scleroderma, patients had lower frequencies of complicated autoimmune diseases than those in western countries. CONCLUSIONS: The male-to-female ratio, frequencies by age group, symptoms and physical findings among patients with PBC were consistent with previous reports in Japan and from other countries. However, positivity of AMA and the frequency of complicated autoimmune diseases were lower among patients in Japan than among those in western countries.

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“…This survey has been performed every 2-3 years by the Intractable Hepatobiliary Disease Study Group for Research on Measures for Intractable Disease, which is supported by Health and Labor Sciences Research Grants in Japan; the protocol of this survey was described by Nakano et al [24]. In brief, questionnaires were sent to 520 institutions throughout Japan to which active members of the Japan Society of Hepatology and the Japanese Society of Gastroenterology belong, and all patients with PBC, both newly diagnosed and followed up in their institutions, were registered.…”

Section: Methodsmentioning

confidence: 99%

“…In the questionnaires, medical history, clinical symptoms at the time of diagnosis, biochemical test findings, clinical stage, histological findings, and prognosis were investigated in newly registered patients. In patients registered in a previous survey, prognosis, the presence or absence of complications, the clinical stage, and biochemical test findings at the final observation were investigated in each survey and were added to the database [24].…”

Section: Methodsmentioning

confidence: 99%