Protein-losing enteropathy (PLE) is a condition in which the body loses too much protein through the intestines and into the stool. Protein is essential for maintaining fluid balance, supporting the immune system, and keeping tissues healthy, so ongoing protein loss can lead to a range of symptoms.
What Causes Protein-Losing Enteropathy?
Protein-losing enteropathy is not a disease on its own. It occurs when an underlying problem causes proteins to leak from the bloodstream into the intestines. Identifying the cause is important because treatment focuses on addressing what is driving the protein loss.
In lymphatic disorders, PLE is most often caused by intestinal lymphangiectasia. This occurs when lymphatic vessels in the intestines become enlarged, blocked, or abnormal, disrupting normal lymphatic flow and allowing protein-rich lymph fluid to leak into the digestive tract.
Who Can Develop Protein-Losing Enteropathy?
PLE can occur in people with complex lymphatic anomalies (CLAs). It is less common in isolated lymphatic malformations (LMs) but may occur when lymphatic vessels in or near the intestines are involved.
Possible Signs and Symptoms
Symptoms of PLE can vary widely and may include:
- Swelling (edema) in the legs, abdomen, or face
- Low albumin levels (hypoalbuminemia) on blood tests
- Poor growth or weight loss, especially in children
- Nutritional deficiencies
- Weakened immune function, increasing the risk of infections
- Fatigue or general weakness
The severity of symptoms differs from person to person.
How Is Protein-Losing Enteropathy Diagnosed?
Diagnosis of PLE typically involves a combination of medical history, laboratory testing, and imaging.
Blood Tests
Blood tests are used to look for:
- Low albumin or total protein levels
- Signs of nutritional deficiencies or immune system effects related to protein loss
Stool Testing
Special stool tests may be used to confirm that protein is being lost through the intestines. These tests help determine whether the digestive tract is the source of protein loss.
Imaging Studies
Imaging may be used to identify underlying lymphatic abnormalities, such as:
- Abnormal lymphatic vessels near the intestines
- Chylous ascites or other fluid collections
MRI is often preferred when lymphatic involvement is suspected because it provides detailed images without radiation.
How Is Protein-Losing Enteropathy Treated?
Treatment focuses on reducing protein loss and managing the underlying lymphatic problem. Care is individualized and may include:
- Nutritional support, including high-protein diets or supplements
- Specialized dietary approaches when appropriate
- Management of lymphatic complications, such as chyle leaks
- Medications or targeted therapies to support lymphatic function
- Monitoring and treatment of nutritional and immune deficiencies
Long-Term Management of PLE
Protein-losing enteropathy often requires ongoing monitoring and follow-up care. Long-term management focuses on controlling symptoms, preventing complications, supporting nutrition and immune health, and maintaining overall quality of life.
