Treatment algorithm

Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups: see disclaimer

ONGOING

all patients

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treatment of underlying chronic liver disease and prevention of superimposed hepatic insult

As cirrhosis is the pathological end-stage of any chronic liver disease, it is essential to treat the underlying causative condition.

Oral direct-acting antivirals are considered a first-line treatment for chronic hepatitis C virus infection. One systematic review and meta-analysis found that early treatment with direct-acting antivirals reduced the recurrence of hepatocellular carcinoma, liver decompensation, and all-cause mortality, and prevented hepatocellular carcinoma in patients with compensated cirrhosis and without cirrhosis but did not prevent liver transplantation.[93]​ Regimens depend on the genotype and presence or absence of cirrhosis.[94] Local guidance should be consulted. See Hepatitis C. Antivirals may be indicated for patients with chronic hepatitis B.[84][95]​ See Hepatitis B.​

Superimposed hepatic insult may be prevented through the avoidance of alcohol and other hepatotoxic drugs (e.g., non-steroidal anti-inflammatory drugs [NSAIDs] and high doses of paracetamol [>2-3 g/day]), immunisation against hepatitis A and B for susceptible patients, management of metabolic risk factors, maintenance of adequate nutrition, and regular exercise.

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monitoring for complications

Treatment recommended for ALL patients in selected patient group

Cirrhosis is associated with serious complications including portal hypertension causing ascites (further complicated by spontaneous bacterial peritonitis and hepatic hydrothorax), gastro-oesophageal varices and portosystemic encephalopathy, acute kidney injury and hepatopulmonary syndromes, portopulmonary hypertension, hepatocellular carcinoma, and acute-on-chronic liver failure.

Prompt detection and treatment of these complications is essential in order to minimise related morbidity and mortality. Imaging tests include: abdominal ultrasound for the detection of ascites; upper gastrointestinal endoscopy in selected patients for the detection of gastro-oesophageal varices; and abdominal ultrasound, computed tomography (CT), or magnetic resonance imaging (MRI) for the detection of hepatocellular carcinoma.

Other specialised tests may be required depending on individual symptoms.

See Oesophageal varices, Spontaneous bacterial peritonitis, Hepatic encephalopathy, Hepatorenal syndrome, and Hepatocellular carcinoma.

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supportive and palliative care

Treatment recommended for ALL patients in selected patient group

It is recommended that patients hospitalised with cirrhosis receive formal dietician assessment, and steps should be taken to minimise the fasting period prior to procedures (e.g., by giving them a pre-bedtime snack or early-morning snack if the procedure will be in the late afternoon).[138] Parenteral supplementation may be required for those who cannot meet their nutritional intake needs orally. Recommended protein intake for adults with cirrhosis is 1.2 to 1.5 g/kg (ideal body weight) per day, and 1.2 to 2 g/kg (ideal body weight) per day if critically ill.

As cirrhosis is a life-limiting condition, palliative care should be offered alongside other curative therapies, and may be relevant for patients with compensated cirrhosis and decompensated cirrhosis.[174] The American Gastroenterological Association and the American Association for the Study of Liver Disease have both published guidelines on palliative care in patients with cirrhosis. These promote advanced care planning, assessment and management of symptoms (including pain, breathlessness, muscle cramps, sexual dysfunction, insomnia, daytime sleepiness, fatigue, pruritus, anxiety, and depression), screening for carer needs, and early liaison with local palliative care teams.[174][175] Particular note is given to chronic pain management in the setting of diminished liver function and novel treatments for refractory ascites.[175] 

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sodium restriction and diuretic therapy for ascites

Additional treatment recommended for SOME patients in selected patient group

Ascites is the most common complication of cirrhosis.

Every patient with new-onset ascites should undergo a diagnostic paracentesis: cell count with differential, albumin, and total protein should be measured in the ascitic fluid. Ascitic fluid should also be sent for culture and cytology.


Abdominal paracentesis animated demonstration
Abdominal paracentesis animated demonstration

Demonstrates how to perform diagnostic and therapeutic abdominal paracentesis.


The serum-ascites albumin gradient (SAAG) should be calculated: a SAAG of ≥11 g/L with low ascitic fluid total protein is consistent with portal hypertension secondary to cirrhosis.

Treatment involves a no-added-salt diet (daily intake of not more than 5 to 6.5 g) and the use of diuretics.[101]​ First-line diuretic should be spironolactone due to its effects on aldosterone and maintaining normal serum potassium. Furosemide may be added to patients who do not respond to spironolactone.[101]​ Once-daily dosing is typically preferred. The doses of both oral diuretics can be increased simultaneously every 3-5 days (maintaining the 100 mg to 40 mg ratio) if weight loss and natriuresis are inadequate.[176] Serum sodium, potassium, and creatinine should be monitored.

Non-steroidal anti-inflammatory drugs (NSAIDs), ACE inhibitors, and other nephrotoxins should be avoided in patients with ascites.[90]

Primary options

spironolactone: 100 mg orally once daily initially, titrate as needed every 3-5 days, maximum 400 mg/day

OR

spironolactone: 100 mg orally once daily initially, titrate as needed every 3-5 days, maximum 400 mg/day

and

furosemide: 40 mg orally once daily initially, titrate as needed every 3-5 days, maximum 160 mg/day

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liver transplantation

Patients who develop complications of cirrhosis such as hepatocellular carcinoma or signs of decompensation (ascites, jaundice, variceal haemorrhage, hepatopulmonary syndrome, hepatic hydrothorax, portal systemic encephalopathy, or hepatorenal syndrome) should be referred for liver transplant evaluation without delay. Transplant assessment may be a prolonged process and early referrals are preferable. Some patients with acute-on-chronic liver failure may benefit from early liver transplantation.[167]

Orthotopic liver transplantation remains the only curative treatment option for patients with decompensated cirrhosis.

A survival benefit from undergoing liver transplantation is seen when the Model of End-Stage Liver Disease (MELD) score is ≥15. [ MELDNa scores (for liver transplantation listing purposes, not appropriate for patients under age 12 years) (SI units) Opens in new window ]

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transjugular intrahepatic portosystemic shunt (TIPS)

Patients not suitable for liver transplantation should be considered for TIPS placement.[103]

Meta-analyses indicate that TIPS is more effective than paracentesis for control of refractory ascites, but is associated with a higher incidence of hepatic encephalopathy.[103][104][105][106][107][108][109]​ Overall mortality does not appear to differ between the two interventions, but TIPS may confer a modest benefit with respect to transplant-free survival.[103][104][105][106][107][108][109]​ One Cochrane systematic review and network meta-analysis reported that the overall certainty (quality) of evidence was very low, primarily because of unclear or high risk of bias in trials assessing interventions for the treatment of refractory ascites in patients with cirrhosis.[103] [ Cochrane Clinical Answers logo ]

Disease severity at the time of TIPS placement is an important predictive factor for outcomes.[112]

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Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups. See disclaimer

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