Purpose of review: Primary biliary cholangitis (PBC) is a rare autoimmune liver disease that increasingly is diagnosed at early stages of disease. Although survival has improved, a majority suffers from one or more symptom complexes associated with PBC. Much remains unknown about the mechanisms, significance, or effective treatment of these symptoms. The present review will outline the latest understanding and management of the most common symptoms of PBC.
Recent findings: Patients rate fatigue as the most impactful on their quality of life, yet its specificity to PBC remains unsettled. Both central and peripheral nervous system mechanisms have been proposed, but remain enigmatic. Lysophosphatidic acid and G-protein-coupled bile acid receptor 1 have emerged as potential targets for cholestatic-specific pathways of pruritus. Fibrates have also shown promise as a new class of antipruritic in cholestatic patients. PBC and Sjogren's syndrome share many demographic and histological features, but the clinical significance of their association is not well understood.
Summary: Common symptoms in PBC carry an increasing share of the disease burden as patients live longer, yet existing therapies are frequently ineffective or poorly tolerated. Targeted therapies may emerge as the molecular pathways of PBC-associated symptoms are unraveled.