Guidelines on the diagnosis and management of primary biliary cholangitis
image: In 2015, the Chinese Society of Hepatology and the Chinese Society of Gastroenterology published a consensus on primary biliary cholangitis (PBC). In the past years, numerous clinical studies have been published in the field of PBC. To guide the clinical diagnosis and management of PBC patients, the Chinese Society of Hepatology invited a panel of experts to assess the new clinical evidence and formulate the current guidelines.
Credit: Jidong Jia, Xiaoyuan Xu, Hong You, Weijia Duan
Primary biliary cholangitis (PBC), previously referred to as primary biliary cirrhosis, is an autoimmune disorder characterized by intrahepatic cholestasis. While the precise etiology and pathogenesis remain incompletely understood, it is believed to arise from intricate interactions between genetic susceptibility and environmental factors, culminating in immune-mediated damage to the biliary epithelial cells. Predominantly afflicting middle-aged women, PBC manifests most commonly through fatigue and pruritus. Diagnostic laboratory findings typically include elevated serum alkaline phosphatase and gamma-glutamyl transpeptidase, the presence of antimitochondrial antibodies, and increased immunoglobulin M levels. Histologically, the disease is marked by non-suppurative destructive interlobular cholangitis. The cornerstone of PBC treatment is ursodeoxycholic acid (UDCA).
In 2015, the Chinese Society of Hepatology, in conjunction with the Chinese Society of Gastroenterology, released a consensus statement on the diagnosis and management of PBC. Since then, a wealth of clinical evidence has emerged in the field. To refine the clinical approach to PBC diagnosis and patient care, the Chinese Society of Hepatology convened a panel of experts to evaluate the latest clinical findings and to develop updated guidelines. These recommendations are structured in accordance with the Grading of Recommendations Assessment, Development, and Evaluation system.
Over the subsequent years, a significant number of clinical studies have contributed to the growing body of knowledge on PBC. To enhance the clinical diagnosis and management of patients with PBC, the Chinese Society of Hepatology assembled a panel of experts to scrutinize the emerging clinical evidence and to craft the present guidelines.
The guidelines summarize the epidemiology, natural history, clinical manifestations, examinations, histological features, and treatments of PBC, etc. The following gaps and future perspectives are proposed.
- Epidemiological Data: There remains a significant gap in population-based epidemiological studies on PBC within China, which is crucial for understanding the disease burden and distribution.
- Diagnostic and Treatment Criteria: Evidence-based diagnostic criteria and treatment protocols for special conditions, such as autoimmune hepatitis overlap and preclinical PBC, are still insufficient and require further development.
- Second-line Therapy: There is a pressing need for safe and effective second-line therapies for patients who exhibit a suboptimal biochemical response to UDCA, particularly those with compensated or decompensated cirrhosis.
- Etiology and Pathogenesis: Further research is essential to elucidate the etiology and pathogenesis of PBC, with a focus on identifying the initiating factors that provoke the autoimmune response against intrahepatic bile duct epithelium.
- Novel Therapeutic Agents: The development of novel therapeutic agents that target the key pathogenic mechanisms of PBC is urgently required to advance new drug discovery and improve patient outcomes.
Future research directions should focus on addressing the gaps in epidemiological data, establishing evidence-based criteria for special conditions, developing safe second-line therapies for UDCA non-responders, elucidating the etiology and pathogenesis of PBC, and advancing novel therapeutic agents targeting key disease mechanisms.
Full text
https://www.xiahepublishing.com/2310-8819/JCTH-2022-00347
The study was recently published in the Journal of Clinical and Translational Hepatology.
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