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

Abstract

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.

Underestimated Male Prevalence of Primary Biliary Cholangitis in China: Results of a 16-yr cohort study involving 769 patients

“…The most common EHA comorbidity was SS (19.4%), followed by autoimmune thyroid diseases (3.6%) and SLE (3.4%). These percentages were lower than in previous studies, which have reported prevalences of 13.5–36.2%, 4.4–14.4%, and 1.5–3.7% 10, 27–29 , respectively. These differences might be attributable to case-finding methods and under-diagnosing as some reports from Asia found that the prevalence of EHA was much lower 16, 23 .…”

Primary Biliary Cirrhosis (PBC), PBC Autoantibodies, and Hepatic Parameter Abnormalities in a Large Population of Systemic Sclerosis Patients

“…PBC often occurs in association with a variety of other autoimmune conditions5–7. Since its first description in 19707–9, a number of studies have found a high frequency of SSc in patients with PBC, estimating its prevalence at 2% to 18.2%5–7,9–14. These studies also indicated that PBC was most highly correlated with limited SSc and more specifically with anticentromere12,13,15.…”

Anti-neutrophil Cytoplasmic Antibody (ANCA)-associated Vasculitis Associated with Primary Biliary Cirrhosis: A Case Report and Literature Review

“…However, few of these patients had vasculitis. According to a cross-sectional study conducted in 5,000 patients with PBC, concurrent connective tissue diseases mainly consisted of SS (13.5%), RA (7.3%), and SSc (2.0%), although the detection rate of AMAs was high in patients with SSc and low in those with RA or SS (8). However, we cannot discount the possibility of patient differences in autoantibody reactivity to some mitochondrial proteins in those with PBC complicated by connective tissue diseases, including vasculitis, and those with PBC alone.…”