QuestionSBA
QuestionChoice
HIV-associated nephropathy is a disease of the kidneys caused by infection with HIV. It presents with a nephrotic syndrome picture; however, this would not explain this patient's jaundice or palmar erythema. This patient is also currently taking antiretroviral treatment, which is protective against developing HIV-associated nephropathy
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d
HIV-associated nephropathy
QuestionChoice
Chronic and excessive use of alcohol can damage hepatic tissue and lead to hepatitis and eventually cirrhosis. However, in this case, the patient is adamant that they do not drink alcohol. Patients are of course not always honest about their intake of alcohol. However, this patient has a history of intravenous drug use with a co-diagnosis of HIV. These are risk factors for hepatitis C, and therefore at this stage, this is the most likely diagnosis
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Harmful alcohol use
QuestionChoice
This patient is presenting clinically with chronic liver disease, most likely cirrhosis. This is indicated primarily by their examination findings. They have ascites and peripheral oedema, with excessive water retention likely causing them to gain weight. Heart failure, renal failure, and liver failure can all present with peripheral oedema and ascites; however, only liver failure would present with jaundice and palmar erythema. This patient doesn't report drinking alcohol, they have evidence of intravenous drug use, and it is likely they are not using clean needles as they also have a diagnosis of HIV (although it is possible this is not related to their IV drug use). This supports the diagnosis of viral hepatitis. Acute hepatitis B infection leads to chronicity in about 5% of adults, whereas acute hepatitis C infection leads to chronicity in 75% of adults. Hepatitis C is also the most common viral hepatitis found in the UK. This makes hepatitis C infection the most likely diagnosis
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Chronic hepatitis C
QuestionChoice
Although this is a typical picture of liver cirrhosis and this patient has multiple risk factors for viral hepatitis, hepatitis C is the most common viral hepatitis in the UK and is also more likely to become chronic than hepatitis B. Therefore, hepatitis C is the more likely diagnosis. It is important to note that although the rate of chronicity is low in adults (around 5%), it is significantly higher in children (up to 90%)
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Hepatitis B
QuestionChoice
Heart failure can present with ascites, peripheral oedema and hepatomegaly (a consequence of congestion of the systemic vasculature due to right heart failure). However, this would not explain why this patient is jaundiced and has palmar erythema. In combination with clear risk factors for hepatitis C infection, this makes hepatitis C the most likely diagnosis
33585
c
Right sided heart failure
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24317
User
subscriber
Migraine Body
User
subscriber
Migraine Body
QuestionComment
Good explaination
28189
User
subscriber
Embolism Prone
Concept
Chapter
# Summary

Cirrhosis refers to chronic liver disease where there is irreversible scarring of the liver with loss of normal hepatic architecture. The key signs and symptoms vary based on if the cirrhosis is compensated or decompensated, ranging from fatigue and anergia to ascites and jaundice. Investigations include blood tests and imaging or invasive procedures, with the Child-Pugh score used to estimate the severity of cirrhosis. Management strategies involve nutritional guidance, medication, and possibly a liver transplant for severe cases.

# Definition

Cirrhosis refers to **irreversible** scarring of the liver with loss of normal hepatic architecture.

# Epidemiology

The prevalence and incidence of cirrhosis are dependent on the underlying aetiological factors prevalent in the population. Alcohol and viral hepatitis, particularly Hepatitis B and C, are major contributors globally. Non-alcoholic fatty liver disease is increasingly becoming a significant cause due to rising obesity rates.

# Aetiology


- Commonest causes:
  - **Alcohol**
  - **Hepatitis B and C**
  - **Non-alcoholic fatty liver disease (NAFLD)**
- Less common causes:
  - Autoimmune:
    - Autoimmune hepatitis
    - Primary biliary cirrhosis
    - Primary sclerosis cholangitis
    - Sarcoid
  - Genetic:
    - Haemochromatosis
    - Wilson's disease
    - Alpha-1-antitrypsin deficiency
  - Drugs:
    - Methotrexate
    - Amiodarone
    - Isoniazid
  - Others:
    - Budd-Chiari Syndrome
    - Heart failure
    - Tertiary syphilis

# Signs and Symptoms

Clinical features of cirrhosis can be divided based on whether the cirrhosis is compensated or decompensated.

## Compensated Cirrhosis

- Fatigue and anergia
- Anorexia and cachexia
- Nausea or abdominal pain
- Spider naevi
- Gynaecomastia
- Finger clubbing
- Leuconychia
- Dupuytren's contracture
- Caput medusae
- Splenomegaly

[lightgallery]

## Decompensated Cirrhosis

In addition to the symptoms of compensated cirrhosis:

- Ascites and oedema
- Jaundice
- Pruritus
- Palmar erythema
- Gynaecomastia and testicular atrophy
- Easy bruising
- Encephalopathy/confusion
- Liver 'flap'

[lightgallery1][lightgallery2]

# Differential Diagnosis

- Hepatic steatosis: Characterised by the accumulation of fat in the liver, leading to hepatomegaly, mildly elevated serum aminotransferases, and possible right upper quadrant pain. If managed early these changes are reversible, however if not managed there will be progression to cirrhosis.
- Chronic hepatitis: Presents with fatigue, malaise, low-grade fever, and hepatomegaly.
- Congestive heart failure: Symptoms include dyspnoea, fatigue, fluid retention, and peripheral oedema.


# Investigations

Initial blood tests for cirrhosis should include:

- Liver Function Tests: AST, ALT, ALP, GGT, Albumin, and Bilirubin
- Full blood count to look for leucocytosis, thrombocytopaenia, and anaemia 
- Urea and electrolytes to establish baseline renal function and look for hepato-renal syndrome or any electrolyte abnormalities 
- INR to look for coagulopathy
- A low platelet count, elevated aspartate aminotransferase (AST): alanine transaminase (ALT) ratio, high bilirubin, low albumin, or increased prothrombin time or international normalized ratio (INR) can suggest impaired liver function. Patients can however also present with normal blood tests which is why clinical examination findings are equally important.
- Specific tests to determine the potential cause such as Hepatitis serology, cytomegalovirus serology, iron studies, α-1 anti-trypsin, caeruloplasmin level, and auto-antibodies

Imaging and invasive investigations may include:

- Peritoneal tap for microscopy and culture if ascites is present
- Doppler ultrasound to identify Budd-Chiari syndrome
- Transient elastography (fibroscan) or acoustic radiation force impulse imaging should be done for patient groups at risk of cirrhosis: those with hepatitis C infection, increased alcohol intake (men who drink >50 units of alcohol/week, women who drink >35 units of alcohol/week), and those with known alcohol-related disease
	- If cirrhosis is not diagnosed on initial testing, these patients should have retesting for cirrhosis every 2 years
- Liver biopsy for confirmation of diagnosis if in doubt

### Child-Pugh Score

The severity of liver cirrhosis can be estimated by calculating the Child-Pugh score. This scoring system is shown in the table below:

| Score                                | 1    | 2     | 3      |
| ------------------------------------ | ---- | ----- | ------ |
| Bilirubin (umol/l)                   | <34  | 34-51 | >51    |
| Albumin (g/l)                        | >35  | 28-35 | <28    |
| Prothrombin time (seconds prolonged) | <4   | 4-6   | >6     |
| Encephalopathy                       | none | mild  | marked |
| Ascites                              | none | mild  | marked |

The scores are added and the degree of cirrhosis is classified as Child-Pugh A (<7 points), B (7-9 points) or C (>9 points). The score can be used as a predictor of mortality, and may also be used to predict the need for a liver transplant.

### MELD Score

- The Model for End-Stage Liver Disease (MELD) score evaluates the severity of liver disease based on bilirubin, creatinine, and INR levels. 
- Higher scores indicate a greater risk of mortality within a three-month period. MELD scores are used to prioritise patients for liver transplantation. 
- In liver cirrhosis, MELD scores ≥15 typically indicate significant disease severity, with increased mortality rates and the need for liver transplantation consideration.


|MELD Score|Interpretation|
|---|---|
|6-9|Low risk of mortality; Class I (less than 10%)|
|10-19|Intermediate risk of mortality; Class II (19%)|
|20-29|High risk of mortality; Class III (52%)|
|30-39|Severe risk of mortality; Class IV (71%)|
|≥40|Very severe risk of mortality; Class V (95%)|

# Management

Conservative: 

- Ensuring good nutrition and total alcohol abstinence
- Avoiding non-steroidal anti-inflammatory drugs, sedatives, and opiates
- 6 monthly ultrasound scans and serum α-fetoprotein tests for hepatocellular carcinoma detection
- Upper gastrointestinal endoscopy surveillance for oesophageal varices may be needed at diagnosis (one-off) and every 3 years, unless the person is taking carvedilol or propranolol non-selective beta-blocker therapy for primary prevention of decompensation, or for primary prevention of bleeding from medium or large varices

Medical: 

- Using cholestyramine for pruritus
- Managing ascites with fluid restriction, low-salt diet, pharmacological management with spironolactone; furosemide, therapeutic paracentesis, and albumin infusions in severe cases
- Reducing recurrent episodes of encephalopathy through prophylactic lactulose and rifaximin
- Using prophylactic antibiotics in patients at high-risk of spontaneous bacterial peritonitis 

Surgical:

- Liver transplantation is the only definitive management for liver cirrhosis, and different scoring criteria are used to assess severity and suitability for transplanation
- If there is acute liver failure, the King's criteria is used (below):

Paracetamol Toxicity:

- **pH** - < 7.3 at any time or < 7.30 - 7.35 after fluid resuscitation
- **Prothrombin time (INR)** - > 100 seconds or > 6.5
- **Grade 3 or 4 encephalopathy**

Non-Paracetamol-Induced Fulminant Hepatic Failure:

- **INR** - > 50 seconds or > 3.5 despite Vitamin K treatment
- **Serum Creatinine** - > 300 µmol/L
- **Grade 3 or 4 encephalopathy**


- If there is chronic liver failure leading to advanced cirrhosis, UK End-stage Liver Disease (UKELD) score is used to predict disease severity and is based on the MELD score but also includes sodium


# Complications

## Ascites

This results from portal hypertension and hypoalbuminaemia. It gives rise to other complications such as spontaneous bacterial peritonitis.

## Spontaneous bacterial peritonitis (SBP)

Sudden peritonitis can occur in patients with ascites. It may present atypically (often with no abdominal tenderness or guarding) and should be suspected in patients who deteriorate suddenly with no other obvious cause. An ascitic tap with neutrophils >250mm³ indicates SBP. Patients with a low ascitic albumin are especially at risk and should be treated with prophylactic antibiotics.

## Liver failure

With its sequelae of hepatic encephalopathy, jaundice and coagulopathy. The former can result in cerebral oedema progressing to raised intracranial pressure and death, and the latter can contribute to life-threatening bleeds in those with a source of bleeding due to thrombocytopaenia.

## Hepatocellular carcinoma

Patients with cirrhosis are at significantly increased risk, especially those with Hepatitis B and C.

## Oesophageal varices ± haemorrhage

The development of portal hypertension in cirrhosis leads to dilatation of oesophageal veins. These are liable to rupture and this can be fatal, especially in patients with coagulopathy. There can also be gastric varices, and caput medusae (dilatation of umbilical veins) – these are due to portosystemic collaterals trying to get around the cirrhotic liver.

## Renal failure

Cirrhosis and ascites with renal failure is known as hepatorenal syndrome if other causes of acute kidney injury have been ruled out. Abnormal haemodynamics in liver disease cause renal vasoconstriction which makes the kidneys more susceptible to injury. It has a very poor prognosis.




# NICE Guidelines

[Click here to see information on NICE CKS about cirrhosis](https://cks.nice.org.uk/topics/cirrhosis/)
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An example appearance of ascites.
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A 52-year-old woman presents to her GP as she has gained weight over the last eight months. On examination, she has yellow sclera, track marks in the antecubital fossae, reddened palms, abdominal distension with shifting dullness, hepatomegaly and bilateral ankle oedema. Auscultation of the chest is normal. She has a past medical history of HIV diagnosed ten years ago, for which she takes antiretroviral therapy. She regularly uses heroin, but she does not drink.

What is the most likely diagnosis?
a