Changes in disease characteristics of primary biliary cholangitis: An observational retrospective study from 1982 to 2016

Abstract

Aim Disease characteristics of primary biliary cholangitis have changed recently. However, detailed studies on the subject have been limited. Therefore, we aimed to clarify disease characteristics of patients with recent primary biliary cholangitis using the cohort from Niigata University and 21 affiliated hospitals. Methods Overall, 508 patients were enrolled in this study from 1982 to 2016, divided into three cohorts according to their year of diagnosis: ≤1999, 2000–2009 and ≥2010. We compared differences in clinical characteristics, response to ursodeoxycholic acid and prognosis. Results The male‐to‐female ratio increased incrementally from 1:16.4 (≤1999) to 1:3.8 (≥2010) (P < 0.001). In women, the median age at diagnosis increased incrementally from 54.0 years (≤1999) to 60.5 years (≥2010) (P < 0.001) and serum albumin decreased gradually (P = 0.001), which might have affected the increase in the Fibrosis‐4 Index and albumin–bilirubin score. The ursodeoxycholic acid response rate according to the Barcelona criteria increased incrementally from 26.7% (≤1999) to 78.4% (≥2010) (P < 0.010), and those according to other criteria (Paris‐I, Rotterdam and Toronto) were approximately ≥80% in all cohorts. Ten‐year survival rate in the ≤1999 and 2000–2009 cohorts were 98.6% and 95.6%, respectively. These earlier cohorts were also characterized by a higher rate of asymptomatic state and mild histology (83.5% [≤1999] and 84.7% [2000–2009], and 93.6% [≤1999] and 91.1% [2000–2009]). Conclusions Patients with primary biliary cholangitis were characterized by older age at diagnosis and an increase in male to female ratio as well as higher response rates of ursodeoxycholic acid and longer survival, resulting from the early recognition of primary biliary cholangitis.

Cumulative risk of developing a new symptom in patients with primary biliary cholangitis and its impact on prognosis

“…Although this disease mostly affects middle-aged females, the percentage of males is gradually increasing. 1 A Japanese study reported that from 1980 to 1998, 69.9% of patients with PBC were asymptomatic during diagnosis, and the percentage of initially symptomatic patients who had a total Figure 1 Strategy of this study. A total of 483 with primary biliary cholangitis were enrolled.…”

“…This retrospective multicenter analysis included all incident and prevalent alive cases of definite or probable PBC recorded between 1 January 1982, and 31 December 2014 in Niigata University Hospital and a related hospital. The original data in this study were the same as that previously reported by us 1 . The diagnosis of PBC was made based on the criteria established by the Intractable Hepato‐Biliary Diseases Study Group of Japan 12 .…”

“…The original data in this study were the same as that previously reported by us. 1 The diagnosis of PBC was made based on the criteria established by the Intractable Hepato‐Biliary Diseases Study Group of Japan. 12 We excluded patients aged <18 years; diagnosed with autoimmune hepatitis overlap syndrome, UDCA treatment before diagnosing PBC, and other liver diseases; manifesting initial symptom including pruritus, edema, jaundice, ascites, hepatic encephalopathy, or varices; and followed up within 12 months (Fig.…”

“…Recent advances in examination include the development of an antimitochondrial antibody, which is a sensitive serological hallmark of PBC, and its treatment approach is ursodeoxycholic acid (UDCA) therapy. Although this disease mostly affects middle‐aged females, the percentage of males is gradually increasing 1 …”

“…Although this disease mostly affects middle‐aged females, the percentage of males is gradually increasing. 1 …”