Review article: pathophysiology and management of primary biliary cholangitis

Abstract

SummaryBackgroundPrimary biliary cholangitis (PBC), an immune‐mediated disease characterised by destruction of intrahepatic bile ducts, results in progressive damage to the biliary tree, cholestasis and ultimately advanced liver disease. In the last decade, advances in practice have improved clinical care, driven novel therapeutic options and improved risk stratification tools.AimsTo provide an overview of the disease characteristics of PBC and review a patient‐centred management approach for the clinical team caring for those with PBC.MethodsWe reviewed the current literature and guidelines on PBC with a focus on management and therapies.ResultsA confident diagnosis of PBC is usually made based on serum liver tests and immune serology. Management of PBC should focus on three main ‘process’ pillars: (a) treat and risk‐stratify through use of biochemical and prognostic criteria; (b) manage concurrent symptoms and other associated diseases; and (c) stage disease, monitor progression and prevent complications. With ongoing complexities in management, including a newly licensed therapy (obeticholic acid) and alternative non‐licensed treatments and ongoing clinical trials, discussion with PBC expert centres is encouraged.ConclusionsPBC is a dynamic disease wherein current treatment goals have become appropriately ambitious. Goals of care should prioritise prevention of end‐stage liver disease and amelioration of patient symptom burden for all.

Bicyclol Alleviates Signs of BDL-Induced Cholestasis by Regulating Bile Acids and Autophagy-Mediated HMGB1/p62/Nrf2 Pathway

“…Besides UDCA and obeticholic acid, 24-norursodeoxycholic acid (norUDCA) is also used to treat PSC and cholestasis liver diseases with favorable outcomes ( Fickert et al, 2013 ; Fickert et al, 2017 ). Unfortunately, 40% of patients do not have a satisfactory response to UDCA and are at risk of cirrhosis or non-neoplastic complications ( Leung et al, 2020 ; Wagner and Fickert, 2020 ). The results of the present study demonstrated that BDL could lead to the accumulation of serum TBA and TC which induce a significant liver injury, as evidenced by the increasing of serum ALT, AST, ALP, GGT and hepatic necrosis.…”

Diffuse large B-cell lymphoma in the liver accompanied by primary biliary cholangitis: A rare and difficult-to-diagnose tumor with portal venous thrombosis

“…PBC is an autoimmune disease characterized by destruction of intrahepatic bile ducts which results in progressive damage to the biliary tree, cholestasis and ultimately advanced liver disease [ 14 ]. Patients with autoimmune disorders seem to have an elevated risk of lymphoma, especially non-Hodgkin's lymphoma [ 2 , 3 , [11] , [12] , [13] ].…”

“…Patients with autoimmune disorders seem to have an elevated risk of lymphoma, especially non-Hodgkin's lymphoma [ 2 , 3 , [11] , [12] , [13] ]. For PBC patients, advances in practice have currently improved clinical care, driven novel therapeutic options and developed risk stratification tools [ 14 ], though a number of immunosuppressants may not be associated with clinical benefit [ 2 , 3 ]. The use of more potent immunomodulatory agents may raise concerns over the risk of adverse effects including the development of non-Hodgkin's lymphoma [ 2 , 3 , [11] , [12] , [13] ].…”

Post-Transplant Immunosuppression in Autoimmune Liver Disease

“…PBC is a chronic liver disease, with immune mediated injury to biliary epithelial cells and represents an interaction between genetic and environmental factors [57]. Prevalence ranges from 19.1 (Australia) to 40.2 (USA) cases per million persons.…”