The extrahepatic events of Asian patients with primary biliary cholangitis: A 30-year cohort study

Abstract

The extrahepatic complications of primary biliary cholangitis (PBC) in Asian patients remain elusive. A 30-year cohort study of 150 Taiwanese PBC patients treated with ursodeoxycholic acid (UDCA) was conducted. Patients with alkaline phosphatase levels >1.67 × ULN after 1-year treatment were considered suboptimal responders. At baseline, of 150 patients (mean age: 53.75 years), 128 (85.3%) were females, and 34 (22.8%) had cirrhosis. The cumulative incidences of various incident events were all-cause mortality or liver transplantation: 46.7%; extrahepatic mortality: 24.5%; extrahepatic malignancies: 8.1%; hypertension: 46.2%; dyslipidemia: 44.1%; diabetes: 30.6%; hyperuricemia: 11.2%; acute coronary syndrome: 3.1%; cerebral vascular accident (CVA): 8.9%; autoimmune diseases: 16%; and osteoporosis: 20.9%. The 5- to 20-year cumulative incidences for all-cause mortality or liver transplantation and extrahepatic mortality were 16.2–41.3% and 3.1–11.9%, respectively. Baseline associations were age and alpha-fetoprotein levels with extrahepatic mortality, 80% due to sepsis; age with extrahepatic malignancies and hypertension; gender and hyperuricemia with CVA; and UDCA response with autoimmune disease. Conclusions: Sepsis accounted for most extrahepatic mortality in PBC patients, and the longer the follow-up was, the higher the extrahepatic/all-cause mortality ratio. Baseline age is crucial for incident extrahepatic events and only CVA shows gender-dimorphism; the association between UDCA response and autoimmune disease requires further investigation.

APASL clinical practice guidance: the diagnosis and management of patients with primary biliary cholangitis

“…PBC typically affects middle-aged women, with a female to male ratio as high as 10:1 [ 41 – 44 ]. However, the female to male ratio (3.9–6.2:1) was much lower in recently reported cohorts from Korea [ 11 ], Japan [ 12 ], China Mainland [ 45 ], Hong Kong [ 13 ] and Taiwan [ 46 ].…”

We Are Not Immune: Racial and Ethnic Disparities in Autoimmune Liver Diseases

“…The incidence of PBC globally among Asians has been controversial; previous literature regarded PBC to be uncommon among Asians. ( 26‐28 ) However, according to a recent meta‐analysis, the prevalence of PBC in the Asia‐Pacific region may be greater than previously reported (11.9 cases per 100,000 persons). ( 26 ) The countries with the highest prevalence are Japan and China, with similar prevalence rates to the United States and Europe.…”

Identification of the Representative Primary Biliary Cholangitis Cohort in Taiwan: A Comparison of Four Nationwide Cohorts

“…These differences in outcomes between C1 and C4 suggest that defining PBC with biochemistry measurements and UDCA treatment, in addition to the ICD-9-CM code, increases the representativeness of C4 for PBC patients in Taiwan. Consistent with this finding, C4 had the highest rates of osteoporosis and dyslipidemia, both of which are common extrahepatic complications of PBC [ 7 ]. Moreover, the mortality of C4 (34.22%) was within the reported range of mortality in PBC patients [ 25 ] and supported the reliability of C4 as the representative PBC corhort in Taiwan.…”

“…However, some patients treated with UDCA or OCA do not exhibit adequate responses, as only 60–70% of PBC patients treated with UDCA exhibit complete responses [ 5 ], and up to 60% of UDCA suboptimal responders do not have a therapeutic response to OCA [ 6 ]. The early detection and treatment of PBC is therefore important to prevent complications [ 7 ].…”