Decompensated Cirrhosis

Decompensated cirrhosis is cirrhosis complicated by ascites, variceal bleeding, hepatic encephalopathy or jaundice, with a median survival of about 2 years.

• Causes: chronic hepatitis B and C, alcohol, metabolic associated fatty liver disease, autoimmune and cholestatic liver disease, haemochromatosis, Wilson disease, and cardiac cirrhosis.
• Pathophysiology: portal hypertension, splanchnic vasodilatation, and impaired synthetic function.
• Acute decompensation can follow infection, bleeding, alcohol, drugs, or portal vein thrombosis, and may progress to acute-on-chronic liver failure.

Ascites with abdominal distension, weight gain, peripheral oedema; jaundice; haematemesis or melaena; confusion, sleep reversal, asterixis; fever and abdominal pain (spontaneous bacterial peritonitis); reduced urine output.

• Muscle wasting, spider naevi, palmar erythema, caput medusae, splenomegaly, gynaecomastia, testicular atrophy.
• Differential diagnosis: heart failure, nephrotic syndrome, malignant ascites, tuberculous peritonitis, constrictive pericarditis, Budd-Chiari syndrome, portal vein thrombosis, other causes of encephalopathy (sepsis, uraemia, hypoglycaemia, intracranial bleed).
• Complications: refractory ascites, spontaneous bacterial peritonitis, hepatorenal syndrome, variceal bleeding, encephalopathy, hepatic hydrothorax, hyponatraemia, hepatopulmonary syndrome, portopulmonary hypertension, hepatocellular carcinoma, coagulopathy, infection and sarcopenia.

Full blood count, liver profile, INR, albumin, urea, creatinine, electrolytes (hyponatraemia), glucose, blood cultures when febrile.

• Diagnostic paracentesis in every patient with ascites on admission: neutrophils of 250 per mm3 or more diagnoses spontaneous bacterial peritonitis; serum-ascites albumin gradient 11 g/L or more indicates portal hypertension; culture in blood culture bottles at the bedside.
• Ultrasound with Doppler (portal vein thrombosis, hepatocellular carcinoma), upper endoscopy for varices, chest radiograph, urine microscopy and sodium (hepatorenal syndrome: creatinine rise with no response to 2 days of diuretic withdrawal and albumin 1 g/kg/day, no shock, no nephrotoxins, no structural kidney disease).
• Aetiology work-up if unknown.

Supportive: sodium restriction 2 g (88 mmol) daily, no fluid restriction unless sodium is below 125 mmol/L, protein 1.2 to 1.5 g/kg/day, late evening snack, avoid NSAIDs, aminoglycosides and ACE inhibitors, alcohol abstinence, vaccination.

Ascites:

• Spironolactone 100 mg plus furosemide 40 mg once daily in the morning, increasing every 3 to 5 days to a maximum of 400 mg and 160 mg daily; aim weight loss 0.5 kg daily (1 kg with oedema).
• Tense or refractory ascites: large volume paracentesis with albumin 8 g per litre removed when more than 5 L drained.
• Stop diuretics if sodium is below 125 mmol/L, creatinine rises, or encephalopathy develops.

Spontaneous bacterial peritonitis:

• Cefotaxime 2 g IV every 8 hours or ceftriaxone 2 g IV daily for 5 days, plus albumin 1.5 g/kg on day 1 and 1 g/kg on day 3.
• Prophylaxis after an episode: norfloxacin 400 mg daily or ciprofloxacin 500 mg daily; primary prophylaxis if ascitic protein below 15 g/L with renal or liver failure.

Variceal bleeding:

• Terlipressin 2 mg IV every 4 hours (or octreotide infusion), ceftriaxone 1 g daily, endoscopic band ligation within 12 hours; secondary prophylaxis with carvedilol 6.25 mg once daily increasing to 12.5 mg daily plus banding.

Hepatic encephalopathy:

• Treat the precipitant; lactulose 30 mL every 2 hours until bowels open, then 15 to 30 mL two to three times daily titrated to 2 to 3 soft stools; add rifaximin 550 mg twice daily for recurrence.

Hepatorenal syndrome:

• Stop diuretics; albumin 1 g/kg (maximum 100 g) on day 1, then 20 to 40 g daily; terlipressin 0.85 mg IV every 6 hours (increase to 1.7 mg every 6 hours at day 3) or infusion 2 mg daily, or noradrenaline infusion 0.5 to 3 mg/h in intensive care.

Other: hepatic hydrothorax (diuretics, avoid chest drain), pruritus (cholestyramine 4 g up to three times daily), hyponatraemia (stop diuretics, albumin, fluid restriction to 1 L daily for sodium below 125 mmol/L), cramps (baclofen 5 to 10 mg three times daily).

Procedures: transjugular intrahepatic portosystemic shunt for refractory ascites or recurrent variceal bleeding; liver transplantation evaluation at first decompensation; palliative care for those not eligible.

Special groups: elderly and frail (nutrition and physiotherapy), pregnancy (specialist care), hepatitis B (tenofovir or entecavir).

Red flags: hypotension, rising creatinine, encephalopathy grade 3 or 4, gastrointestinal bleeding, fever with abdominal pain, sodium below 120 mmol/L.

Follow-up: review every 4 to 8 weeks with sodium, creatinine and weight; hepatocellular carcinoma surveillance every 6 months; vaccination against hepatitis A and B, pneumococcus and influenza.