Chronic liver disease is responsible for most of the clinical burden of liver disease. Chronic liver injury can occur via a variety of mechanisms, including sterile inflammation and activation of innate and adaptive immunity. Despite the diversity of disease aetiologies and the ability of the liver to regenerate, a significant minority of patients with chronic liver disease proceed to liver fibrosis and eventually cirrhosis, which is defined histologically by regenerative hepatocyte nodules surrounded by fibrous bands of matrix. Ongoing liver injury stimulates the development of a myofibroblast cell type which is responsible for matrix remodelling, haemodynamic changes, and immune cell regulation. This typically results in repair without significant modification of the basic liver structure. In a few subjects, this repair process results in alterations of the basic structure of the liver with loss of hepatocyte mass, deposition of collagen, and the development of hypertension in the portal venous system. Although cirrhosis is well defined histologically, there is a spectrum of severity. In early cirrhosis, patients are asymptomatic but with increasing derangement in hepatic function and portal hypertension, patients can decompensate and develop ascites, coagulopathy, encephalopathy, jaundice, renal failure, oesophageal varices, and spontaneous bacterial infections. Management is focused on removing or reducing ongoing liver injury, and managing cirrhosis-related complications by the use of low-salt diets, diuretics, β-blockers, endoscopic therapy, vasopressors, and antibiotics. There is, as yet, no definite role for antifibrotic medications.
Clinical Features of Cystic Fibrosis Patients with Chronic Liver Disease in Turkish National Cystic Fibrosis Registry