What is the treatment for type 2 hepatorenal syndrome?

Treatment of Type 2 Hepatorenal Syndrome

The first-line treatment for type 2 hepatorenal syndrome (HRS-CKD) is focused on managing refractory ascites, with liver transplantation being the definitive treatment. 1

Understanding Type 2 Hepatorenal Syndrome

Type 2 hepatorenal syndrome (now termed HRS-CKD) is characterized by:

• Stable, less severe kidney dysfunction with slower progression compared to HRS-AKI (formerly Type 1)
• Serum creatinine >133 μmol/L or 1.5 mg/dL
• Absence of identifiable kidney pathology
• Association with refractory ascites

Treatment Algorithm

First-Line Management:

1. Management of Refractory Ascites:

   • This is the primary focus for HRS-CKD patients
   • Careful fluid and electrolyte management
   • Consider discontinuation of diuretics upon diagnosis of acute kidney injury 1
   • Consider withholding non-selective beta-blockers, particularly in hypotensive patients

2. Transjugular Intrahepatic Portosystemic Shunt (TIPS):

   • More applicable for HRS-CKD than for HRS-AKI
   • Can improve renal function and control ascites
   • Contraindicated in severe liver failure or significant encephalopathy 1

Pharmacological Treatment:

Unlike HRS-AKI, vasoconstrictors plus albumin are not routinely recommended for HRS-CKD due to high recurrence after withdrawal 1

However, when pharmacological treatment is considered:

1. Terlipressin plus Albumin:

   • Terlipressin 0.85 mg IV every 6 hours
   • Can be titrated based on serum creatinine response
   • Albumin 1 g/kg on day 1, followed by 20-40 g/day
   • Continue until serum creatinine decreases below 1.5 mg/dL or maximum 14 days 1, 2
   • Important safety note: Monitor for respiratory failure, which occurred in 14% of patients in clinical trials 2

2. Alternative Regimens (if terlipressin unavailable):

   • Midodrine + octreotide + albumin:
     • Midodrine titrated up to 12.5 mg orally three times daily
     • Octreotide targeted at 200 μg subcutaneously three times daily
     • Albumin 10-20 g/day IV for up to 20 days 1, 3
   • Norepinephrine plus albumin (in ICU setting) 1

Definitive Treatment:

Liver transplantation is the only curative treatment for hepatorenal syndrome 1

• Expedited referral for transplantation should be considered for all patients
• Simultaneous liver-kidney transplantation may be considered for patients with significant kidney damage or those who have been under prolonged renal support therapy (>12 weeks)

Monitoring and Precautions

1. Respiratory Monitoring:

   • Assess oxygenation saturation before initiating terlipressin
   • Do not initiate terlipressin in patients with hypoxia (SpO2 <90%)
   • Monitor oxygen saturation continuously during treatment
   • Discontinue terlipressin if SpO2 decreases below 90% 2

2. Cardiovascular Monitoring:

   • Terlipressin can cause cardiovascular or ischemic complications in approximately 12% of patients
   • Contraindicated in patients with ongoing coronary, peripheral, or mesenteric ischemia 1, 2

3. Volume Status Assessment:

   • Patients with fluid overload may be at increased risk of respiratory failure
   • Consider reducing or discontinuing albumin administration in volume-overloaded patients 2

Prognosis

The prognosis for HRS patients remains poor despite treatment:

• Median survival of approximately 3 months for all HRS patients
• Early diagnosis and treatment are critical for improving outcomes
• Patients with serum creatinine <5.0 mg/dL are more likely to benefit from terlipressin 1, 2

Important Caveats

1. Limitation of terlipressin use: Patients with a serum creatinine >5 mg/dL are unlikely to experience benefit from terlipressin 2

2. Diagnostic confirmation: Ensure proper diagnosis by excluding other causes of renal failure such as hypovolemia, shock, parenchymal renal diseases, and nephrotoxic drugs 1

3. Prevention: Treatment of spontaneous bacterial peritonitis (SBP) with albumin plus antibiotics reduces the risk of HRS development 1