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]
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 symptoms of (disease name) usually develop in the first/ second/ third decade of life, and start with symptoms such as ___.
- The symptoms of (disease name) typically develop ___ years after exposure to ___.
- If left untreated, [#]% of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
- 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.
Complications
- The high mortality rate associated with cirrhosis is primarily due to complications.
- Common complications of cirrhosis include:
- Complications due to portal hypertension include:
- Ascites : Ascites is the most common complication of cirrhosis
- Due to increased pressure, fluid leaks through the vasculature into the abdominal cavity
- Esophageal varices: Increased pressure in the portal vein leads to collateral portal blood flow through vessels in the stomach and esophagus
- Portal vein thrombosis
- Easy bruising and bleeding - due to the decreased production of coagulation factors
- Jaundice
- Itching (pruritus)
- Hepatic encephalopathy : due to inability of the liver to clear ammonia and related nitrogenous substances from the blood, which are carried to the brain
- The features of hepatic encephalopathy are as follows:
- Changes in sleep pattern (insomnia and hypersomnia)
- Unresponsiveness
- Forgetfulness
- Poor concentration
- asterixis
- Coma
- The features of hepatic encephalopathy are as follows:
- Sensitivity to drugs due to decreased metabolism in the liver
- Ascites : Ascites is the most common complication of cirrhosis
- Hepatocellular carcinoma is primary liver cancer and is most commonly seen in cirrhotic patients with:
- Infection: due to immune system dysfunction
- Spontaneous bacterial peritonitis: Infection of the fluid in the abdomen due to ascites with intestinal bacteria
- Symptoms and signs include pain, tenderness and altered mental status
- patient may be asymptomatic in early stages
- Findings on diagnostic paracentesis such as a positive culture and/or absolute neutrophil count >250/mm3 are confirmatory
- 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.
- Complications due to portal hypertension include:
- 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
- Prognosis is generally excellent/good/poor, and the 1/5/10-year mortality/survival rate of patients with [disease name] is approximately [#]%.
- Depending on the extent of the [tumor/disease progression/etc.] at the time of diagnosis, the prognosis may vary. However, the prognosis is generally regarded as poor/good/excellent.
- The presence of [characteristic of disease] is associated with a particularly [good/poor] prognosis among patients with [disease/malignancy].
- [Subtype of disease/malignancy] is associated with the most favorable prognosis.
- The prognosis varies with the [characteristic] of tumor; [subtype of disease/malignancy] have the most favorable prognosis.
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
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.