Cirrhosis of the liver is a chronic illness that progresses at a variable rate, dependent on the etiology and the activity of the offending toxin. It is a dynamic process, which is potentially reversible in the earlier stages if the offending agent is either removed or modified. Classically, cirrhosis has been defined histologically as architecturally abnormal nodules separated by bands of fibrous tissue. The diagnosis has usually been based on clinical suspicion, confirmed by radiologic studies and a liver biopsy as the gold standard. However, sampling error on biopsy may lead to incorrect staging of the disease, where the transition from severe fibrosis to cirrhosis is misinterpreted. Therefore, advanced chronic liver disease has been proposed as a more encompassing definition for the transitional stages. However, in this chapter, we will continue to use cirrhosis as the term to characterize this entity.
Patients with cirrhosis may be further classified as compensated or decompensated. Decompensation is defined as the appearance of ascites, variceal bleeding, hepatic encephalopathy or jaundice. A prognostic classification consisting of five stages has been proposed to account for the increasing mortality with progressive decompensation (Figure 160-1). Stage one includes patients with compensated cirrhosis that do not have any complications such as ascites or esophageal varices. In stage two, patients have developed nonbleeding esophageal varices but are still considered compensated. Stage three describes patients who have transitioned to decompensation, characterized by the development of hemorrhage from esophageal varices. Patients may further progress to stage four which is characterized by the development of nonbleeding complications, including the development of ascites, hepatic encephalopathy or the hepatorenal syndrome. Stage five includes patients who have developed a second decompensating event. In a recent study by D’Amico et al that included 494 patients with alcoholic, viral or cryptogenic cirrhosis, the initial decompensating event was ascites (33%), variceal bleeding (10%), and hepatocellular carcinoma (9%), while encephalopathy or jaundice as the first decompensating event was rare. In this study, the 5-year mortality was 1.5% for patients in stage one compared to 88% for patients in stage five. In addition to describing the natural history of the disease process, a potential benefit of this staging system is the ability to target specific therapy based on the prognosis at a given stage. For example, patients who presented with variceal bleeding without ascites had a significantly better survival than those in whom ascites was present at the onset of the bleeding event and would be less likely to be considered as candidates for liver transplantation.
Schematic representation of 5-year transitioning rate across stages and to death for the whole series of patients. Arrows represent transitions and the numbers close to each arrow are the relevant transition rates. A fairly steady increase in death rate was found across stages. (From D’Amico G, et al. Competing risks and prognostic stages of cirrhosis: a 25-year inception cohort study of 494 patients. Aliment Pharmacol Ther. 2014;39(10):1180-1193.)