Management of primary biliary cholangitis: results from a large real-life observational study in France and Belgium

Abstract

Background and aims: To assess the characteristics, care, treatment response, and outcomes of primary biliary cholangitis (PBC) patients recently followed-up by hepato-gastroenterologists in various French and Belgian healthcare settings. Methods: This retrospective cohort study included patients with PBC who recently visited 79 hepato-gastroenterologists in France and Belgium. Data were collected at the time of diagnosis and at last visit and were compared according to biochemical response (BR) to ursodeoxycholic acid (UDCA) (BR), using Paris I–II criteria, and clinical outcomes. Results: A total of 436 patients (mean age at diagnosis 57 years, 88% females, median follow-up 5.2 years) were included. Liver biopsy, transient elastography, or none of these two procedures were performed at baseline in 216 (50%), 194 (45%), and 107 (25%) patients, respectively. Late-stage disease (histological stage III or IV, or transient elastography ≥9.6 kPa, or bilirubin >17 µM and albumin <35 g/L, or platelets <150.000/µl, or unequivocal signs of portal hypertension or cirrhosis) was reported in 37% of patients. UDCA was taken by 95% of patients (27% had suboptimal dosage). Inadequate BR was observed in 37% of patients. Clinicians overestimated disease control. Liver-related complications occurred in 9% of patients. Bilirubin and albumin independently predicted inadequate BR; advanced disease stage and inadequate BR independently predicted complications. Conclusions: Recently followed-up French and Belgian patients with PBC had homogeneous management. Late stage at diagnosis and inadequate BR were reported in around 40% of patients. Disease control was frequently overestimated by clinicians. Disease stage and BR were the main prognostic factors.

Noninvasive evaluation of fibrosis in adult biliary diseases

“…Another large study, this time across France and Belgium, investigated data from two time points, the date of diagnosis and last visit, of 436 patients with PBC followed by hepatologists with good experience with PBC [9 ▪ ]. This study showed that the evaluation of the disease stage was quite often incomplete (in 25% of patients, neither liver biopsy nor LSM was assessed at baseline) and nonresponsiveness to ursodeoxycholic acid (UDCA) according to Paris criteria frequently went unrecognized by clinicians who therefore overestimated disease control.…”

Distinct signatures of gut microbiota and metabolites in primary biliary cholangitis with poor biochemical response after ursodeoxycholic acid treatment

“…Primary biliary cholangitis (PBC) is a chronic cholestatic immune liver disease characterized by persistent cholestasis, interlobular bile duct damage, liver fibrosis, eventual cirrhosis, and death [ 1 ]. Its prevalence in Europe has an overall rate of 22.27 cases per 100,000 inhabitants and a pooled incidence rate of 1.7 new cases per 100,000 inhabitants per year, with a female predominance (1.6–4.8:1 female: male ratio) [ 2 ].…”

“…The main characteristics of PBC are cholestasis caused by abnormal bile acids (BAs) metabolism and interlobular bile duct damage caused by abnormal immune response [ 1 ]. Studies had suggested the critical role of microbiome by interacting with BAs metabolism and immunity.…”

Primary biliary cholangitis: molecular pathogenesis perspectives and therapeutic potential of natural products

“…Primary biliary cirrhosis (PBC) is clinically a type of autoimmune cholestatic liver disease characterized by serum autoantibody antimitochondrial antibodies (AMAs) ( 1 , 2 ), chronic progressive cholestasis, interlobular bile duct damage, liver inflammation, eventual cirrhosis, and death ( 3 , 4 ). Studies have found that the incidence and prevalence of PBC have increased globally in the past few decades ( 5 , 6 ), especially among women ( 7 ).…”