What is the significance of elevated Angiotensin-Converting Enzyme (ACE) levels?

From the Guidelines

Elevated Angiotensin-Converting Enzyme (ACE) levels are significant as they are associated with sarcoidosis, a complex disease with manifold clinical manifestations, and can indicate more aggressive disease and multiple-organ involvement. The clinical significance of high ACE levels is highlighted in the context of sarcoidosis diagnosis and management, as seen in the european position paper on rhinosinusitis and nasal polyps 2020 1. According to this study, serum ACE is the most widely used laboratory test for the investigation of sarcoidosis, and elevated serum levels of ACE are usually associated with more aggressive disease and multiple-organ involvement.

Clinical Implications

The diagnosis and detection of sarcoidosis, as outlined in the American Journal of Respiratory and Critical Care Medicine 1, also emphasizes the importance of ACE levels in the diagnostic process. Elevated ACE levels can be a supportive feature of sarcoidosis, particularly when considered in conjunction with other clinical findings and tests.

Key Points

• Elevated ACE levels are associated with sarcoidosis and can indicate more aggressive disease and multiple-organ involvement 1
• ACE levels are a useful tool in the diagnosis of sarcoidosis, particularly when considered in conjunction with other clinical findings and tests 1
• The significance of high ACE levels underscores the importance of considering sarcoidosis in the differential diagnosis of patients with elevated ACE levels, and prompt further investigation or consideration of treatment options.

Management Considerations

In the context of real-life clinical medicine, elevated ACE levels should prompt further investigation to rule out sarcoidosis and other conditions associated with high ACE levels. This may involve a combination of clinical evaluation, imaging studies, and laboratory tests to determine the underlying cause of the elevated ACE levels.

From the Research

Significance of Elevated Angiotensin-Converting Enzyme (ACE) Levels

Elevated ACE levels can be indicative of various conditions, including:

• Granulomatous diseases such as sarcoidosis, as seen in studies 2, 3
• Pneumocystis carinii pneumonia (PCP), with significantly elevated ACE levels found in patients with PCP 4
• Cardiovascular diseases, with elevated ACE expression in tissues associated with increased risk 5
• Genetic mutations, with certain mutations leading to increased circulating ACE activity 6

Conditions Associated with Elevated ACE Levels

The following conditions have been associated with elevated ACE levels:

• Sarcoidosis: Elevated serum ACE activity is found in 60-70% of sarcoidosis patients 2, 3
• Pneumocystis carinii pneumonia (PCP): Significantly elevated ACE levels found in patients with PCP, particularly in those who smoke 4
• Familial hyperactivity of ACE: A rare, benign condition characterized by extremely elevated ACE activity, which can be confirmed by genetic testing 2
• Granulomatous diseases: Increased circulating ACE activity has been reported in multiple granulomatous disorders 6

Clinical Significance of ACE Phenotyping

ACE phenotyping, which includes determination of ACE activity and quantification of ACE immunoreactive protein levels, can help:

• Identify patients with various mutations in ACE 5
• Detect the presence of ACE inhibitors in the blood 5
• Establish normal values for ACE levels in healthy populations 5
• Advance precision medicine screening techniques by establishing different risk groups based on ACE phenotype 5