Protein-Losing Enteropathy

Protein-losing enteropathy (PLE) is a condition where you lose too much protein through your intestines — essentially, by pooping it out. It can happen if you have a chronic (long-lasting) disease that damages your intestinal lining. When your intestinal lining is damaged, proteins in your blood can leak through the weakened walls and enter your GI tract. Once inside, they travel through and all the way out.

Your body is made up of thousands of proteins that each have important jobs. They help build your cells and tissues and allow them to grow and repair. They support your immune system, gene function and more. But with protein-losing enteropathy, you lose proteins that your body systems need to function. The result is low protein in your blood and overall protein deficiency, which affects you in many ways.

Symptoms of protein-losing enteropathy

Symptoms of protein-losing enteropathy can include:

• Abdominal pain
• Bloated stomach
• Chronic diarrhea
• Fever
• Frequent illness
• Swelling in your face, arms and legs
• Unexplained weight loss or growth faltering

You may also have other symptoms related to the underlying cause.

Protein-losing enteropathy causes

In PLE, a weakness in your intestinal walls allows proteins in your blood to leak into your intestines. Once inside, these proteins follow the path of your food through the long, winding tube until it comes out at the end. Healthcare providers have identified three categories of disorders that can cause protein-losing enteropathy. Most of these are gastrointestinal (GI) diseases. But some affect your intestines indirectly.

Causes include:

Primary erosive gastrointestinal disorders

These conditions damage your intestinal lining, creating weaknesses or openings for protein to leak in. They include:

• Cancer in your digestive system
• Carcinoid syndrome
• Graft vs. host disease
• Inflammatory bowel diseases (like ulcerative colitis and Crohn’s disease)
• Peptic ulcer disease
• Pseudomembranous colitis

Non-erosive gastrointestinal disorders

These conditions disrupt or rearrange the cells in your intestinal walls, which can lead to protein loss. They include:

• Amyloidosis
• Celiac disease
• Eosinophilic gastritis
• HIV/AIDS
• Intestinal parasites
• Lupus
• Mixed connective tissue disease
• Whipple’s disease

Disorders affecting your lymph system

These conditions can cause protein-rich lymph to leak into your intestines through lymph channels connected to them.
They include:

• Congenital heart disease
• Constrictive pericarditis
• Fontan procedure
• Liver disease
• Lymphoma
• Retroperitoneal fibrosis
• Right-sided heart failure
• Sarcoidosis

Complications of protein-losing enteropathy

The most abundant protein in your blood is albumin. When albumin is low, fluids leak into your bloodstream, causing swelling. In severe cases, fluid can collect around your lungs and around your heart. Albumin also helps transport vitamins, minerals and hormones through your body. Without it, you can develop severe malnutrition. In children, this can cause growth and developmental delays.

How doctors diagnose this condition

To diagnose your condition, your healthcare provider will:

1. Ask about your symptoms and medical history
2. Conduct a physical exam
3. Order blood work to test for abnormal protein levels and other signs
4. Look for possible causes of low protein in your blood

Additional tests might include:

• Stool test: Your provider might ask for a sample of your poop to test for proteins. High levels of a protein called alpha-1-antitrypsin (A1AT) can confirm a diagnosis of protein-losing enteropathy.
• Endoscopy: Your provider might need to look inside your intestines with an endoscope to find the source of the leak or diagnose the cause. Sometimes, they can treat it at the same time.
• Lymphangiography: Your provider might need to examine your lymphatic system to locate the cause of protein-losing enteropathy. They can sometimes fix the issue during the exam.

What is the treatment for protein-losing enteropathy?

Treatment of protein-losing enteropathy involves:

1. Treating the underlying condition that’s causing it
2. Restoring your protein levels and overall nutrition

Treating the underlying condition might involve medications and/or procedures. If your provider finds the cause during an endoscopy or lymphangiography exam, they can sometimes treat it then. For example, they might be able to remove a blockage or seal a wound. Some causes are curable, and others might need long-term treatment. Your provider will discuss your treatment plan with your diagnosis.

To restore your nutrition, a registered dietitian will design a personalized medical nutrition therapy plan for you. Some people might need treatment in the hospital to receive nutrition through a tube or through a vein. When you‘re ready to go home, you‘ll have guidance on what foods to eat and supplements to take. You‘ll need to eat lots of high-protein foods to meet your protein requirements.

What’s the outlook for protein-losing enteropathy?

Most people start to feel better after beginning treatment for protein-losing enteropathy and the condition causing it. If your condition is curable, your symptoms may go away altogether. If you have a long-term condition, you may need lifelong treatment. But with treatment, most conditions are manageable. Your healthcare provider can tell you more about what to expect from your condition.