What are the causes of Acute Respiratory Distress Syndrome (ARDS) in patients with cirrhosis (Liver Cirrhosis)?

From the Guidelines

ARDS in patients with cirrhosis can develop from several specific causes related to their liver disease, including bacterial infections, sepsis, aspiration pneumonia, and pulmonary vascular complications such as hepatopulmonary syndrome and portopulmonary hypertension, as noted in the 2024 AASLD practice guidance on acute-on-chronic liver failure and the management of critically ill patients with cirrhosis 1.

Causes of ARDS in Cirrhosis

The primary causes of ARDS in cirrhotic patients include:

• Bacterial infections, particularly spontaneous bacterial peritonitis and pneumonia, which trigger inflammatory responses that damage lung tissue
• Sepsis, a common pathway to ARDS in these patients, as their compromised immune function makes them vulnerable to overwhelming infections
• Aspiration pneumonia, which occurs frequently due to altered mental status from hepatic encephalopathy
• Acute-on-chronic liver failure, which can precipitate ARDS through systemic inflammation and cytokine release
• Hepatopulmonary syndrome and portopulmonary hypertension, pulmonary vascular complications of cirrhosis that may contribute to or mimic ARDS
• Fluid overload from hypoalbuminemia and portal hypertension, which can worsen lung function through pulmonary edema
• Transfusion-related acute lung injury following blood product administration, to which cirrhotic patients have heightened susceptibility

Management Considerations

Management should focus on treating the underlying cause while providing supportive care with careful fluid management, as these patients have poor outcomes once ARDS develops due to their limited physiological reserve, as highlighted in the 2024 AASLD practice guidance on acute-on-chronic liver failure and the management of critically ill patients with cirrhosis 1. Key considerations include:

• Investigation and treatment of coexisting pulmonary comorbidities related to cirrhosis
• Use of high-flow nasal cannula (HFNC) therapy and lung protective ventilation with low tidal volume and low positive end-expiratory pressure (PEEP) in patients with acute hypoxemic respiratory failure
• Therapeutic thoracentesis or paracentesis in patients with respiratory compromise related to hydrothorax or tense ascites
• Palliative care consultation to improve outcomes and reduce healthcare utilization in critically ill patients with cirrhosis.

From the Research

Causes of ARDS in Cirrhosis

• Advanced liver disease is associated with hypoxemia and respiratory failure by various mechanisms, including hepatopulmonary syndrome, portopulmonary hypertension, and hepatic hydrothorax 2
• Critically ill cirrhotic patients are prone to episodes of decompensation requiring intensive care unit admission and management, which can lead to acute respiratory distress syndrome (ARDS) 2
• Sepsis, acute-on-chronic liver failure, and shock on intensive care unit admission are risk factors for ARDS development in patients with cirrhosis 3
• The etiology of cirrhosis or alcohol use are not associated with ARDS development 3

Mechanisms and Outcomes

• ARDS is a prognostic factor for mortality in patients with liver cirrhosis admitted to the ICU, with a 28-day mortality or liver transplantation rate of 54% in patients with mechanical ventilation and 66% in patients with ARDS 4
• The prognosis of cirrhotic patients with ARDS appears related to extrapulmonary organ dysfunctions rather than pulmonary dysfunction 5
• Hospital mortality is higher in cirrhotic patients with ARDS compared to those without ARDS, with a mortality rate of 82.2% in ARDS patients and 27.6% in non-ARDS patients 3
• Early recognition and treatment for infection might be important for improving the high mortality in this group of patients 3

Clinical Characteristics

• Patients with ARDS and cirrhosis are more frequently female, with suspected infection, and have higher mean Model for End-Stage Liver Disease and Chronic Liver Failure-Sequential Organ Failure Assessment scores than patients without ARDS 6
• The incidence of ARDS within the first 72 hours of admission is 8% in critically ill patients with liver cirrhosis 4
• One out of ten critically ill cirrhotic patients develop ARDS within 72 hours after admission 4