Cirrhosis natural history, complications and prognosis
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Shankar Kumar, M.B.B.S. [2]] Aditya Govindavarjhulla, M.B.B.S. [3]
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Overview
Cirrhosis is an irreversible process, the course of which is highly variable from patient to patient. The natural history progresses so that there is a lengthy stage of compensation, followed by development of complications and sequelae as a result of the cirrhosis. The devastating complications include complete liver failure or the development of hepatocellular carcinoma. Other complications are portal hypertension, ascites, jaundice, itching, esophageal varices, spontaneous bacterial peritonitis, hepatic encephalopathy, hepatorenal syndrome, hepatopulmonary syndrome and cardiomyopathy. Prognosis depends on the causes, existing complications and a variety of factors which make prediction of life expectancy questionable. There are scores by which to classify severity and to determine suitability for liver transplant.
Natural History
The general course of cirrhosis is characterized by a long stage of compensation, which can be followed by deterioration and development of specific complications. Life threatening complications can develop in almost any patient. Once the first complication in a patient with cirrhosis is seen, it is soon followed by a string of subsequent complications which significantly decreases life expectancy.
It is difficult to predict the exact course of the disease and generalize it to the entire population. Several factors play a key role such as an individual's existing hepatic function, the etiology of cirrhosis, whether the ongoing damage can be halted or slowed down and whether the patient develops hepatocellular carcinoma.
Various issues need to be addressed in addition to formulating the life expectancy of a cirrhotic patient. It is important to consider whether the patient can withstand the chosen therapeutic intervention, and whether the intervention would significantly improve the outcome.
Even though the course of cirrhosis is dependent on multiple factors, there is a definite need for prognostic models and scoring systems especially when it comes to management with liver transplantation.
Decompensated Cirrhosis
In patients with previously stable cirrhosis, decompensation may occur due to various causes, such as constipation, infection (of any source), increased alcohol intake, medication, bleeding from esophageal varices or dehydration. It may take the form of any of the complications of cirrhosis listed above.
Patients with decompensated cirrhosis generally require admission to the hospital, with close monitoring of the fluid balance, mental status, and emphasis on adequate nutrition and medical treatment - often with diuretics, antibiotics, laxatives and/or enemas, thiamine and occasionally steroids, acetylcysteine and pentoxifylline. Administration of saline is generally avoided as it would add to the already high total body sodium content that typically occurs in cirrhosis.
Complications
As the disease progresses, complications may develop. In some people, these may be the first signs of the disease. The high mortality rate associated with cirrhosis is actually due to these complications.
- Portal hypertension - blood normally carried from the intestines and spleen through the hepatic portal vein flows slower and the pressure increases; this leads to the following complications:
- Ascites - The most common complication of cirrhosis begins with portal hypertension. Due to increased pressure, fluid leaks through the vasculature into the abdominal cavity. Note here that the patients who DO NOT have portal hypertension DO NOT develop ascites.
- Esophageal varices - collateral portal blood flows through vessels in the stomach and esophagus. These blood vessels may become enlarged and are more likely to burst. Before the advent of current therapies, the mortality rate was as high as 30% after a single episode.
- Portal vein thrombosis
- Easy bruising and bleeding - due to the decreased production of coagulation factors.
- Jaundice - due to the decreased processing of bilirubin.
- Itching (pruritus) due to bile products deposited in the skin.
- Hepatic encephalopathy - the liver does not clear ammonia and related nitrogenous substances from the blood, which are carried to the brain. The very first features to appear are changes in sleep pattern (insomnia and hypersomnia) which can be followed by worsening in the form of neglect of personal appearance, unresponsiveness, forgetfulness, trouble concentrating, and asterixis.
- Sensitivity to medication due to decreased metabolism of the active compounds.
- Hepatocellular carcinoma is primary liver cancer and it is most commonly seen in cirrhotic patients with Hepatitis B or C, non-alcoholic steatohepatitis and hemochromatosis. Its diagnosis is most often delayed. It should be suspected when decompensation occurs in a previously compensated patient, for example, a RISING AFP and not simply a high AFP should raise suspicion.
- Infection - Cirrhosis can cause immune system dysfunction, leading to infection. Signs and symptoms of infection may be non-specific and are more difficult to recognize (e.g. worsening encephalopathy but no fever).
- Spontaneous bacterial peritonitis: Fluid in the abdomen (ascites) may become infected with bacteria normally present in the intestines. Suspect this in end stage liver disease. Patient may have pain, tenderness and altered mental status or can be asymptomatic. There should be a low threshold for a diagnostic paracentesis. A positive culture and/or absolute neutrophil count >250/mm3 is confirmatory. There is high mortality if treatment is delayed.
- Hepatorenal syndrome - insufficient blood supply to the kidneys, causing acute renal failure. This complication has a very high mortality (over 50%). This is NOT a new disease rather it is the state of least perfusion to the kidneys. It can be masked clinically because there is decreased muscle mass and hepatic urea synthesis, so there would be little elevation of BUN and creatinine. It is a diagnosis of exclusion (exclude causes of renal dysfunction first). It carries a poor prognosis unless attempts are made to improve the hepatic function.
- Pulmonary diseases associated with cirrhosis include--
- Hepatopulmonary syndrome - presents as a triad comprised of existing liver disease, increased alveolar-arterial gradient and intra-pulmonary vascular dilatations. It has a prevalence of 4-47%. Note here that mild hypoxemia can also be present due to ascites with the intra-abdominal fluid pressing on the diaphragm. [1]
- Hepatic hydrothorax - The intra-abdominal fluid can seep in through the diaphragm into the pleural space (mostly right sided) leading to a pleural effusion.
- Portopulmonary hypertension - There is increased blood pressure over the lungs as a consequence of portal hypertension.[1]. The prevalence is 2% in patients with cirrhosis. It is diagnosed with echocardiography and catheterization but treatment with medical therapy or liver transplant is less likely curative.
- Cardiomyopathy - Cirrhotic cardiomyopathy has 50% prevalence in cirrhotic patients. It presents with normal or increased cardiac output at rest but notably decreases in stress conditions.
- Muscle cramps - These are thought to occur due to reduced circulating plasma volume, though exact cause is not fully understood.
Prognosis
| Well-Compensated, no alcohol | 35% mortality at 2 years |
| Onset of Ascites | 50% mortality at 2 years |
| Variceal bleeding | 65% mortality at 1 year (35% short-term mortality) |
Scoring Systems
- Model for End-Stage Liver Disease
- Pediatric End-Stage Liver Disease
- Child-Pugh score
- Child-Turcotte classification
The severity of cirrhosis is commonly classified with the older Child-Turcotte score and the newer Child-Pugh score. This score uses bilirubin, albumin, INR, presence and severity of ascites and encephalopathy to classify patients in class A, B or C; class A has a favorable prognosis, while class C is at a high risk of death. It was devised in 1964 by Child and Turcotte and modified in 1973 by Pugh et al.[2]
More modern scores, used in the allocation of liver transplants but also in other contexts, are the Model for End-Stage Liver Disease (MELD) score and its pediatric counterpart, the Pediatric End-Stage Liver Disease (PELD) score.
References
- ↑ 1.0 1.1 Rodriguez-Roisin R, Krowka MJ, Herve P, Fallon MB; ERS Task Force Pulmonary-Hepatic Vascular Disorders (PHD) Scientific Committee. Pulmonary-Hepatic vascular Disorders (PHD). Eur Respir J 2004;24:861-80. PMID 15516683.
- ↑ Pugh RN, Murray-Lyon IM, Dawson JL, Pietroni MC, Williams R. Transection of the oesophagus for bleeding oesophageal varices. Br J Surg 1973;60:646-9. PMID 4541913.