What is the treatment approach for Hepato(hepatic) Renal Syndrome in patients with advanced liver disease, typically cirrhosis?

From the Guidelines

The treatment of Hepatorenal Syndrome (HRS) in patients with advanced liver disease focuses on improving renal perfusion and addressing the underlying pathophysiology, with the first-line treatment combining vasoconstrictors with albumin to increase effective arterial blood volume and improve renal function, as recommended by the most recent study 1. The goal of treatment is to improve renal function and reduce mortality.

• The preferred vasoconstrictor therapy is terlipressin (0.5-2mg IV every 4-6 hours for up to 14 days) combined with albumin (20-40g/day), as it has been shown to improve renal function and reduce mortality in patients with HRS 1.
• In regions where terlipressin is unavailable, norepinephrine (0.5-3 mg/hour continuous infusion) with albumin or midodrine (7.5-12.5mg orally three times daily) plus octreotide (100-200mcg subcutaneously three times daily) with albumin are alternatives, as they have also been shown to be effective in improving renal function in patients with HRS 1.
• Albumin is typically administered at 1g/kg on day one, followed by 20-40g daily, as it helps to increase effective arterial blood volume and improve renal function 1.
• Diuretics should be discontinued, and nephrotoxic medications avoided, as they can worsen renal function in patients with HRS.
• Volume status should be optimized with careful fluid management, as volume overload can lead to complications such as pulmonary edema.
• For patients who don't respond to medical therapy, liver transplantation remains the definitive treatment, as it addresses the underlying liver dysfunction that causes the splanchnic vasodilation and renal hypoperfusion characteristic of HRS, and has been shown to improve survival in patients with HRS 1.
• Renal replacement therapy may be used as a bridge to transplantation in selected cases, as it can help to improve renal function and reduce mortality in patients with HRS who are awaiting liver transplantation.
• Early diagnosis and prompt treatment are crucial, as HRS carries high mortality if not addressed quickly, with mortality rates ranging from 50-90% if left untreated 1.

From the Research

Definition and Characteristics of Hepato-Renal Syndrome

• Hepato-renal syndrome (HRS) is a form of kidney dysfunction that occurs in liver cirrhosis, characterized by a marked impairment of kidney function in response to circulatory and hemodynamic alterations 2.
• HRS is classified into two forms: acute kidney injury (HRS-AKI) and chronic kidney disease, with HRS-AKI being one of the most severe forms of AKI in patients with cirrhosis 2.
• The syndrome is often triggered by an infection and appears in the setting of advanced decompensated cirrhosis, making differential diagnosis with other causes of AKI crucial 2.

Treatment Approach for Hepato-Renal Syndrome

• The treatment of HRS-AKI is based on the administration of vasoconstrictor drugs in combination with volume expansion with albumin 2.
• Liver transplantation or simultaneous liver-kidney transplantation is considered the ideal definitive treatment for HRS-AKI, with a significant impact on patients' quality of life 2.
• Combination therapy with midodrine, octreotide, and albumin has shown promise in improving renal function and short-term survival in patients with type 1 and type 2 HRS 3, 4.
• The use of vasoconstrictors, such as terlipressin, in combination with albumin, is effective in 40%-50% of patients with type 1 HRS and improves survival 5.

Prognostic Factors and Survival

• The prognosis of HRS-AKI is poor, with median survival ranging from 8 to 49 days depending on the etiology of the underlying liver disease 6.
• Increased serum creatinine and urinary sodium at the initiation of therapy are prognostic factors for mortality in patients with HRS 6.
• The etiology of the underlying liver disease, such as autoimmune or alcoholic liver disease, can influence survival in patients with HRS 6.
• Treatment with octreotide, midodrine, and albumin, as well as the type of HRS, are independently associated with improved survival in patients with HRS 4.