Chapter 12 Primary Biliary Cirrhosis

摘要: Publisher Summary This chapter reviews the epidemiology, pathophysiology, clinical presentations, prognosis, and treatment of Primary Biliary Cirrhosis (PBC). PBC is an autoimmune liver disease characterized by chronic progressive loss interlobular bile ducts. An immune-mediated destruction duct epithelium thought to mediate its pathogenesis. It a that primarily affects middle-aged women all races. epithelial cells drives pathogenesis PBC. only in which autoreactive T B responding E2 subunit pyruvate dehydrogenase complex (PDC-E2) are detected. Persistence fetal maternal circulation may play role The pathophysiological basis seems lie demonstrated defects immune tolerance results activation expansion self-antigen-specific lymphocyte clones production circulating autoantibodies, cytokines, other inflammatory mediators. Fatigue pruritus most common presenting complaints. Additional findings include hyperlipidemia, hypothyroidism, metabolic bone disease, coexisting such as Sjogren's syndrome scleroderma.