What Is Hepatorenal Syndrome?
Hepatorenal syndrome (HRS) is a dangerous, often deadly complication of advanced liver disease. Most people who get it have advanced cirrhosis, or scarring of the liver. ‘Hepato’ refers to the liver. HRS causes sudden kidney failure (also called renal failure), which requires immediate medical care. Without treatment, kidney failure can be fatal within weeks.
Hepatorenal Syndrome Symptoms
If you develop HRS, the symptoms that come with it may not alert you to the fact that your health is at serious risk. Instead, you simply may feel generally unwell or uncomfortable. Such symptoms include:
- Fatigue
- Nausea
- Belly pain
- An unpleasant taste in your mouth
- Less frequent need to pee
Along with these symptoms, however, you also may experience the symptoms of advanced liver disease, such as:
Jaundice. This is a yellowing of your skin and the whites of your eyes, which occurs when you have too much bilirubin in your bloodstream. Bilirubin is a substance created when old red blood cells break down. It’s normal to have some bilirubin in your blood, but a high level is a sign of liver disease.
Ascites. This condition causes an excessive buildup of fluid in your belly. It usually results from cirrhosis, or late-stage liver disease in which the liver has become permanently scarred.
An enlarged spleen. This is an organ located in your abdomen, on its upper left side.
A swollen belly. This can be caused by ascites, an enlarged spleen, or an enlarged liver.
Cognitive problems. These include confusion and difficulties with memory caused by hepatic encephalopathy. This is a condition that develops when your diseased liver can no longer adequately filter toxins out of your blood. As those toxins build up, they begin to affect your brain.
You also may have itchy skin and bruise and bleed more easily than normal, as well as dark urine and light-colored poop.
Types of Hepatorenal Syndrome
HRS can be divided into two types:
HRS-AKI
This is short for hepatorenal syndrome-acute kidney injury. Experts refer to it as acute because the kidney injury it causes can happen quite suddenly. This type of HRS comes in three stages. The stage depends on how much your creatinine has increased.
- Stage 1: creatinine has increased by at least 0.3 milligrams per deciliter (mm/dL) or 1.5 to 2 times from your initial, or baseline, level.
- Stage 2: creatinine has increased 2 to 3 times from your baseline level.
- Stage 3: creatinine has increased at least 3 times from your baseline or serum creatinine has increased to greater than or equal to 4.0 mg/dL with an acute increase greater than or equal to 0.3 mg/dL or initiation of renal replacement therapy.
HRS-NAKI
This stands for hepatorenal syndrome-non-acute kidney injury. It’s used for people with cirrhosis whose kidney injury does not meet the criteria for HRS-AKI.
Causes of Hepatorenal Syndrome
HRS is caused by the narrowing — also called constricting — of the blood vessels in your kidneys. When that happens, your kidneys don’t get an adequate supply of blood and they begin to shut down. That’s called kidney failure.
Why does having liver disease impact the kidneys in this way and lead to HRS? Experts don’t know. But they think that the downstream effects of liver disease somehow trigger that narrowing of the blood vessels. Though the cause remains a mystery, several risk factors appear to play a part in the development of HRS.
Hepatorenal Syndrome Risk Factors
Though the exact causes remain unknown, some conditions — or combinations of related conditions — are suspected of triggering HRS in people with advanced liver disease.
Portal hypertension
This is a type of high blood pressure that affects the portal vein, which delivers blood to the liver from the organs that make up your digestive system, including your:
- Large and small intestine
- Stomach
- Pancreas
- Gallbladder
- Spleen
Cirrhosis of the liver causes most cases of portal hypertension, which affects your blood vessels, widening some while narrowing others, including those in the kidneys, which get narrower as the disease progresses. This narrowing is called renal vasoconstriction.
Spontaneous bacterial peritonitis (SBP)
This is an infection of the two-layer lining of your abdomen, called the peritoneum. SBP can develop if you have ascites, the buildup of fluid in parts of your abdomen, including between the layers of your peritoneum. Ascites frequently develops in people who have cirrhosis, and is a symptom of HRS.
Internal bleeding
Portal hypertension can lead to the breakdown of blood vessels in your abdomen, causing internal bleeding in your belly. HRS may develop as a result of this bleeding.
Certain medications
- Diuretics, prescribed to help your body remove excess fluid, may trigger HRS.
- Nonsteroidal anti-inflammatory drugs (NSAIDs), like aspirin, ibuprofen, and naproxen, reduce blood flow to the kidneys as well as the kidneys’ ability to function normally. Taking these drugs may boost your risk of HRS if you have cirrhosis.
Hepatorenal Syndrome Diagnosis
Diagnosing HRS can be difficult. No single test will tell your doctor that you have it. Instead, you’ll need to undergo a discussion of your medical history and a thorough exam, which may include blood tests, urine tests, and imaging tests. This will help your doctor rule out other possible causes of your symptoms.
In order to receive a diagnosis of HRS, you must meet certain criteria, including:
- You have been diagnosed with cirrhosis and ascites.
- Tests have revealed a significant and rapid rise in your level of creatinine, a waste product normally filtered out of the bloodstream by the kidneys.
- You are not in shock, a dangerous condition that causes a sudden reduction of blood flow in your body. Shock can be a response to trauma, heat stroke, blood loss, and other causes.
- You don’t take and haven’t recently taken drugs known to harm the kidneys, such as NSAIDS, a type of antibiotic called aminoglycosides, and contrast chemicals used in certain imaging exams, such as X-rays and computed tomography (CT) scans.
- There is nothing wrong with the structure of your kidneys as shown by a normal kidney ultrasound.
Hepatorenal Syndrome Treatment
Treatment will first address your liver disease. That’s because your kidneys likely will begin working better once your liver function improves. Often, though, this will mean a liver transplant. That’s because the liver disease usually has advanced too far to do any other treatment.
In some cases, medication may help your liver to function, but current medical guidelines recommend transplant as the most effective treatment for people who remain healthy enough to tolerate a transplant. You should discuss what’s best for you with an experienced care team that includes a liver specialist (hepatologist), a kidney specialist (nephrologist), and a surgeon.
While you wait for a transplant, your doctor will recommend steps to prevent more damage to your kidneys, such as the following:
Stopping certain medications. You should avoid drugs like diuretics and nonsteroidal anti-inflammatory drugs (NSAIDs), like aspirin, ibuprofen, and naproxen.
Albumin therapy. Albumin is a protein that helps improve low blood flow to the kidneys. You receive it via injection.
Hemodialysis. In this procedure, a machine takes over the function of your kidneys to remove waste, salt, and excessive fluids from your blood.
Vasoconstrictor medications. These drugs help to improve blood flow to the kidneys by tightening blood vessels in your body that have become too wide to move a normal amount of blood.
Antibiotics. You’ll take these if you have an infection that’s contributing to your HRS.
IV fluids. This will help blood flow to your kidneys and restore your electrolyte balance. Electrolytes are minerals, like sodium and potassium. At the right levels, these electrolytes support many important functions in your body. Normally, your kidneys work to maintain this balance.
Paracentesis. This is a procedure that removes the excess fluid that has built up in your belly due to ascites.
HRS can damage your kidneys to the point that you will require regular hemodialysis or a kidney transplant even after a liver transplant.
Hepatorenal Syndrome Prevention
HRS most commonly occurs in people with advanced liver disease, or cirrhosis. If you have liver disease, you can lower your chance of developing HRS by following a treatment plan that will keep your liver disease from getting worse or even help your liver get better. Your treatment plan will include important lifestyle changes that will protect the health of your liver, including:
- Avoiding alcohol completely
- Losing weight
- Improving your diet
If your liver disease has progressed to cirrhosis, there is a chance that HRS can develop. However, experts estimate that HRS occurs 1 in 10 people with cirrhosis, so it’s not guaranteed that you will develop it. Unfortunately, your doctor will not be able to predict whether it will happen to you.
One thing that may help reduce your odds of HRS: preventing spontaneous bacterial peritonitis (SBP). This infection of the peritoneum, which lines your stomach, is considered the most common trigger of HRS. About 1 in 4 people who develop HRS had SBP. Your doctor can determine whether you have a high risk of SBP. If you do, antibiotics will help to prevent the infection. This, in turn, may help prevent HRS.
Hepatorenal Syndrome Prognosis
A liver transplant greatly improves your chances of surviving this devastating condition. Approximately 60% of people who have a liver transplant are still alive three years later. Within weeks, most people with HRS will see improvements in their kidney function, though about 1 in 20 will require dialysis on an ongoing basis.
An estimated 50% of people with HRS-AKI will die within three months. The more advanced your liver disease, the more unlikely you are to survive. HRS also can quickly sicken you to the point that a liver transplant would not be safe for you.
Takeaways
Hepatorenal syndrome is a sudden and quickly fatal complication of advanced liver disease, or cirrhosis. It causes the blood vessels in the kidneys to narrow. This limits blood flow and leads to kidney failure. Survival usually requires a liver transplant. Early diagnosis and treatment of liver disease can help prevent HRS by ensuring that your liver disease does not advance to cirrhosis.
Hepatorenal Syndrome FAQs
What is the life expectancy of someone with hepatorenal syndrome?
With a liver transplant, about 60% survive at least three years. An estimated 50% of people with HRS who do not undergo a transplant die within 90 days.
What are the criteria for diagnosis of HRS?
The main criteria are advanced liver disease and ascites and a significant and rapid rise in creatinine in your blood, which indicates kidney damage. Your doctor will do tests to rule out other possible causes, such as being in shock, taking drugs known to harm the kidneys, and bacterial infections.
Is hepatorenal syndrome reversible?
Yes. With a liver transplant, you will likely regain your kidney function within a few weeks. If your kidneys do not recover fully, you may require ongoing dialysis or kidney transplant.
