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Protein-Losing Enteropathy

Updated : March 15, 2024





Background

The disorder known as protein-losing enteropathy (PLE) is characterized by an excessive loss of digestive system proteins. Lower blood protein levels are the consequence of this loss which usually passes through the intestinal mucosa. Due to protein shortage and fluid imbalance, PLE can cause a variety of symptoms and problems.  

PLE may result from several underlying gastrointestinal disorders. Whipple’s disease, intestinal lymphangiectasia, gastrointestinal tract infections, inflammatory bowel disease, celiac disease, cancer, and specific drugs are a few of them. 

Epidemiology

PLE is regarded as an uncommon condition. Due to differences in diagnostic standards, the variety of underlying illnesses is linked to PLE. the dearth of extensive epidemiological studies concentrating on PLE, it is difficult to pinpoint the precise prevalence.   

While PLE can happen to anyone at any age, certain underlying disorders that are linked to it may have a specific age distribution.

Anatomy

Pathophysiology

The barrier function of the intestinal mucosa controls the flow of liquids, proteins and nutrients into the circulation from the intestinal lumen. This barrier may be breached in PLE for several reasons including inflammation, lymphatic blockage, or injury to the intestinal epithelial cells.  

Increased permeability brought on by disruption of the intestinal barrier may permit proteins to seep into the intestinal lumen from the circulation. Either transcellular or paracellular channels between epithelial cells may be the source of this leak. 

 

Etiology

The illness known as intestinal lymphangiectasia is typified by the enlargement and malfunction of the gut wall’s lymphatic vessels. This may be acquired or congenital. While acquired forms can arise from illnesses including lymphatic obstruction, damage to lymphatic vessels, or conditions linked to elevated lymphatic pressure, congenital forms can be caused by genetic abnormalities impacting lymphatic development.  

In those with a genetic predisposition, gluten consumption can result in the autoimmune illness celiac disease. Consuming gluten triggers an immunological reaction that weakens the villous atrophy and hinders the absorption of nutrients, including proteins. 

Genetics

Prognostic Factors

The prognosis is largely dependent on the underlying illness that is producing PLE. In contrast to congenital intestinal lymphangiectasia or systemic lupus erythematosus, illnesses like inflammatory bowel disease or celiac disease may have various prognoses.   

The degree to which protein has been lost into the intestinal lumen is a critical prognostic factor. If treatment is not received, severe and prolonged protein loss might deteriorate prognosis by causing edema, ascites, severe hypoalbuminemia, and nutritional deficits. 

Clinical History

Age Group:  

PLE may occur in infants and children due to congenital or acquired conditions such as congenital intestinal lymphangiectasia, protein intolerance, cow’s milk protein allergy. 

Intestinal lymphangiectasia, a common cause of PLE in children, often presents in infancy or early childhood. 

Physical Examination

  • Vital Signs: Vital signs such as blood pressure, heart rate, respiratory rate, and temperature should be assessed, with particular attention to signs of dehydration. 
  • Edema and Fluid Accumulation: Peripheral edema may be present, particularly in the lower extremities, ankles, or sacral region, due to hypoalbuminemia and fluid retention. 
  • Abdominal Examination: Abdominal examination may reveal tenderness, distension, or palpable masses, depending on the underlying cause of PLE. 
  • Signs of Gastrointestinal Bleeding: Evidence of gastrointestinal bleeding, such as melena or hematochezia, may be observed in some cases of PLE, particularly if there is concurrent intestinal mucosal damage or vascular abnormalities. 
  • Skin Changes: Cutaneous manifestations of PLE may include pallor, ecchymoses, petechiae, or signs of dermatitis or skin breakdown due to malnutrition or edema. 

Age group

Associated comorbidity

Protein loss and hypoalbuminemia can lead to fluid accumulation in the interstitial spaces and abdominal cavity. Edema and ascites are common complications of PLE and can cause discomfort, respiratory compromise, and impaired mobility. 

PLE can lead to malabsorption of nutrients, including proteins, fats, vitamins, and minerals, due to impaired intestinal function. Malnutrition can result in weight loss, muscle wasting, micronutrient deficiencies, and overall nutritional deficits, affecting overall health and quality of life. 

Chronic protein loss and malabsorption in PLE can contribute to the development of anemia. Iron deficiency anemia is common, resulting from impaired absorption of iron in the intestines.  

Associated activity

Acuity of presentation

PLE may present acutely with sudden onset symptoms, especially if the underlying cause is related to an acute gastrointestinal insult or infection. 

Acute presentations may include severe diarrhea, abdominal pain, rapid weight loss, and signs of fluid overload such as ascites or pleural effusions. 

Acute PLE may be triggered by conditions such as gastrointestinal infections, inflammatory bowel disease exacerbations, or acute lymphatic obstruction. 

PLE may also present sub acutely, with symptoms developing over days to weeks rather than suddenly. Subacute presentations may involve a gradual onset of symptoms such as chronic diarrhea, abdominal discomfort, fatigue, and unintended weight loss. 

 

Differential Diagnoses

  • Celiac Disease: Celiac disease is an autoimmune disorder triggered by gluten ingestion, resulting in villous atrophy and malabsorption of nutrients, including proteins. 
  • Intestinal Lymphangiectasia: Intestinal lymphangiectasia is characterized by dilation and dysfunction of intestinal lymphatics, leading to protein-rich lymph leakage into the gastrointestinal tract. 
  • Lymphoma: Lymphoma involving the gastrointestinal tract can cause obstruction or infiltration of lymphatic vessels, leading to protein loss. 
  • Gastrointestinal Infections: Various infectious agents, including bacteria, viruses, and parasites, can cause inflammation and mucosal damage in the gastrointestinal tract, leading to protein loss. 

Laboratory Studies

Imaging Studies

Procedures

Histologic Findings

Staging

Treatment Paradigm

  • Dietary Modifications: Dietary management plays a crucial role in managing PLE. A high-protein diet with adequate caloric intake is recommended to help replenish lost proteins and support nutritional needs. 
  • Nutritional Support: In cases of severe malnutrition or inability to meet nutritional requirements orally or parenteral nutrition may be necessary. Enteral nutrition involves administering liquid nutrients directly into the gastrointestinal tract, while parenteral nutrition provides nutrients intravenously. 
  • Supportive Therapy: Supportive measures may be necessary to manage complications associated with PLE, such as edema, ascites, and electrolyte imbalances.  

by Stage

by Modality

Chemotherapy

Radiation Therapy

Surgical Interventions

Hormone Therapy

Immunotherapy

Hyperthermia

Photodynamic Therapy

Stem Cell Transplant

Targeted Therapy

Palliative Care

Use of non-pharmacological approach for Protein-Losing Enteropathy

  • High-Protein Diet: Consuming a diet rich in high-quality protein sources can help replenish lost proteins and support overall nutritional needs. Lean meats, poultry, fish, eggs, dairy products, legumes, nuts, and seeds are good sources of protein. 
  • Reduced Fat Intake: For conditions associated with fat malabsorption, such as intestinal lymphangiectasia, reducing dietary fat intake may be beneficial.  
  • Balanced Nutrition: Ensuring a balanced diet that provides adequate calories, vitamins, minerals, and other essential nutrients is important for maintaining overall health and supporting the body’s healing processes. 
  • Avoiding Trigger Foods: Identifying and avoiding foods that exacerbate gastrointestinal symptoms, such as spicy or greasy foods, caffeine, alcohol, and high-fiber foods, can help manage symptoms and improve comfort. 
  • Fluid Management: Ensuring sufficient fluid intake is important, especially in cases of diarrhea or fluid losses due to edema or ascites. Encouraging the consumption of water, electrolyte-rich beverages, and oral rehydration solutions can help prevent dehydration. 
  • Physical Activity: Regular physical activity can promote overall health, improve gastrointestinal motility, and support weight management. Encouraging individuals with PLE to engage in low-impact exercises such as walking, swimming, or gentle stretching can be beneficial. 

 

Use of Corticosteroids

  • Prednisone: It exerts potent anti-inflammatory effects by suppressing immune responses and reducing inflammation in the gastrointestinal tract. 

By reducing inflammation and promoting mucosal healing, prednisone can help alleviate symptoms such as diarrhea, abdominal pain, and malabsorption. 

Use of monoclonal antibody

  • Pozelimab: It works by targeting and inhibiting the activity of angiopoietin-like protein 4 (ANGPTL4), which plays a role in the regulation of lymphatic vessel permeability.  

By blocking ANGPTL4, Pozelimab aims to reduce lymphatic leakage and protein loss in patients with PLE. 

Use of Intervention with a procedure in treating Protein-Losing Enteropathy

  • Endoscopy: Upper gastrointestinal (GI) endoscopy and colonoscopy may be performed to visualize the mucosa of the esophagus, stomach, small intestine, and colon. 
  • Balloon Enteroscopy: Balloon-assisted enteroscopy allows for direct visualization of the small intestine using an endoscope with an inflatable balloon at its tip. 
  • Capsule Endoscopy: Capsule endoscopy involves swallowing a small, wireless camera capsule that captures images of the gastrointestinal tract as it passes through the digestive system. 

Capsule endoscopy is particularly useful for evaluating the small intestine, which may be difficult to visualize with traditional endoscopy. 

Use of phases in managing Protein-Losing Enteropathy

  • Initial Assessment and Diagnosis: The first phase involves a comprehensive evaluation to assess the patient’s clinical presentation, medical history, symptoms, and laboratory findings. 
  • Stabilization and Symptom Management: Once a diagnosis of PLE is established, the focus shifts to stabilizing the patient and managing symptoms. 
  • Monitoring and Follow-Up: Ongoing monitoring and follow-up are essential components of PLE management to assess treatment response, monitor disease progression, and adjust therapy as needed. 
  • Long-Term Management and Support: The final phase of PLE management involves long-term monitoring, maintenance therapy, and support to manage the chronic nature of the condition. 

Medication

Media Gallary

Protein-Losing Enteropathy

Updated : March 15, 2024




The disorder known as protein-losing enteropathy (PLE) is characterized by an excessive loss of digestive system proteins. Lower blood protein levels are the consequence of this loss which usually passes through the intestinal mucosa. Due to protein shortage and fluid imbalance, PLE can cause a variety of symptoms and problems.  

PLE may result from several underlying gastrointestinal disorders. Whipple’s disease, intestinal lymphangiectasia, gastrointestinal tract infections, inflammatory bowel disease, celiac disease, cancer, and specific drugs are a few of them. 

PLE is regarded as an uncommon condition. Due to differences in diagnostic standards, the variety of underlying illnesses is linked to PLE. the dearth of extensive epidemiological studies concentrating on PLE, it is difficult to pinpoint the precise prevalence.   

While PLE can happen to anyone at any age, certain underlying disorders that are linked to it may have a specific age distribution.

The barrier function of the intestinal mucosa controls the flow of liquids, proteins and nutrients into the circulation from the intestinal lumen. This barrier may be breached in PLE for several reasons including inflammation, lymphatic blockage, or injury to the intestinal epithelial cells.  

Increased permeability brought on by disruption of the intestinal barrier may permit proteins to seep into the intestinal lumen from the circulation. Either transcellular or paracellular channels between epithelial cells may be the source of this leak. 

 

The illness known as intestinal lymphangiectasia is typified by the enlargement and malfunction of the gut wall’s lymphatic vessels. This may be acquired or congenital. While acquired forms can arise from illnesses including lymphatic obstruction, damage to lymphatic vessels, or conditions linked to elevated lymphatic pressure, congenital forms can be caused by genetic abnormalities impacting lymphatic development.  

In those with a genetic predisposition, gluten consumption can result in the autoimmune illness celiac disease. Consuming gluten triggers an immunological reaction that weakens the villous atrophy and hinders the absorption of nutrients, including proteins. 

The prognosis is largely dependent on the underlying illness that is producing PLE. In contrast to congenital intestinal lymphangiectasia or systemic lupus erythematosus, illnesses like inflammatory bowel disease or celiac disease may have various prognoses.   

The degree to which protein has been lost into the intestinal lumen is a critical prognostic factor. If treatment is not received, severe and prolonged protein loss might deteriorate prognosis by causing edema, ascites, severe hypoalbuminemia, and nutritional deficits. 

Age Group:  

PLE may occur in infants and children due to congenital or acquired conditions such as congenital intestinal lymphangiectasia, protein intolerance, cow’s milk protein allergy. 

Intestinal lymphangiectasia, a common cause of PLE in children, often presents in infancy or early childhood. 

  • Vital Signs: Vital signs such as blood pressure, heart rate, respiratory rate, and temperature should be assessed, with particular attention to signs of dehydration. 
  • Edema and Fluid Accumulation: Peripheral edema may be present, particularly in the lower extremities, ankles, or sacral region, due to hypoalbuminemia and fluid retention. 
  • Abdominal Examination: Abdominal examination may reveal tenderness, distension, or palpable masses, depending on the underlying cause of PLE. 
  • Signs of Gastrointestinal Bleeding: Evidence of gastrointestinal bleeding, such as melena or hematochezia, may be observed in some cases of PLE, particularly if there is concurrent intestinal mucosal damage or vascular abnormalities. 
  • Skin Changes: Cutaneous manifestations of PLE may include pallor, ecchymoses, petechiae, or signs of dermatitis or skin breakdown due to malnutrition or edema. 

Protein loss and hypoalbuminemia can lead to fluid accumulation in the interstitial spaces and abdominal cavity. Edema and ascites are common complications of PLE and can cause discomfort, respiratory compromise, and impaired mobility. 

PLE can lead to malabsorption of nutrients, including proteins, fats, vitamins, and minerals, due to impaired intestinal function. Malnutrition can result in weight loss, muscle wasting, micronutrient deficiencies, and overall nutritional deficits, affecting overall health and quality of life. 

Chronic protein loss and malabsorption in PLE can contribute to the development of anemia. Iron deficiency anemia is common, resulting from impaired absorption of iron in the intestines.  

PLE may present acutely with sudden onset symptoms, especially if the underlying cause is related to an acute gastrointestinal insult or infection. 

Acute presentations may include severe diarrhea, abdominal pain, rapid weight loss, and signs of fluid overload such as ascites or pleural effusions. 

Acute PLE may be triggered by conditions such as gastrointestinal infections, inflammatory bowel disease exacerbations, or acute lymphatic obstruction. 

PLE may also present sub acutely, with symptoms developing over days to weeks rather than suddenly. Subacute presentations may involve a gradual onset of symptoms such as chronic diarrhea, abdominal discomfort, fatigue, and unintended weight loss. 

 

  • Celiac Disease: Celiac disease is an autoimmune disorder triggered by gluten ingestion, resulting in villous atrophy and malabsorption of nutrients, including proteins. 
  • Intestinal Lymphangiectasia: Intestinal lymphangiectasia is characterized by dilation and dysfunction of intestinal lymphatics, leading to protein-rich lymph leakage into the gastrointestinal tract. 
  • Lymphoma: Lymphoma involving the gastrointestinal tract can cause obstruction or infiltration of lymphatic vessels, leading to protein loss. 
  • Gastrointestinal Infections: Various infectious agents, including bacteria, viruses, and parasites, can cause inflammation and mucosal damage in the gastrointestinal tract, leading to protein loss. 
  • Dietary Modifications: Dietary management plays a crucial role in managing PLE. A high-protein diet with adequate caloric intake is recommended to help replenish lost proteins and support nutritional needs. 
  • Nutritional Support: In cases of severe malnutrition or inability to meet nutritional requirements orally or parenteral nutrition may be necessary. Enteral nutrition involves administering liquid nutrients directly into the gastrointestinal tract, while parenteral nutrition provides nutrients intravenously. 
  • Supportive Therapy: Supportive measures may be necessary to manage complications associated with PLE, such as edema, ascites, and electrolyte imbalances.  

  • High-Protein Diet: Consuming a diet rich in high-quality protein sources can help replenish lost proteins and support overall nutritional needs. Lean meats, poultry, fish, eggs, dairy products, legumes, nuts, and seeds are good sources of protein. 
  • Reduced Fat Intake: For conditions associated with fat malabsorption, such as intestinal lymphangiectasia, reducing dietary fat intake may be beneficial.  
  • Balanced Nutrition: Ensuring a balanced diet that provides adequate calories, vitamins, minerals, and other essential nutrients is important for maintaining overall health and supporting the body’s healing processes. 
  • Avoiding Trigger Foods: Identifying and avoiding foods that exacerbate gastrointestinal symptoms, such as spicy or greasy foods, caffeine, alcohol, and high-fiber foods, can help manage symptoms and improve comfort. 
  • Fluid Management: Ensuring sufficient fluid intake is important, especially in cases of diarrhea or fluid losses due to edema or ascites. Encouraging the consumption of water, electrolyte-rich beverages, and oral rehydration solutions can help prevent dehydration. 
  • Physical Activity: Regular physical activity can promote overall health, improve gastrointestinal motility, and support weight management. Encouraging individuals with PLE to engage in low-impact exercises such as walking, swimming, or gentle stretching can be beneficial. 

 

  • Prednisone: It exerts potent anti-inflammatory effects by suppressing immune responses and reducing inflammation in the gastrointestinal tract. 

By reducing inflammation and promoting mucosal healing, prednisone can help alleviate symptoms such as diarrhea, abdominal pain, and malabsorption. 

  • Pozelimab: It works by targeting and inhibiting the activity of angiopoietin-like protein 4 (ANGPTL4), which plays a role in the regulation of lymphatic vessel permeability.  

By blocking ANGPTL4, Pozelimab aims to reduce lymphatic leakage and protein loss in patients with PLE. 

  • Endoscopy: Upper gastrointestinal (GI) endoscopy and colonoscopy may be performed to visualize the mucosa of the esophagus, stomach, small intestine, and colon. 
  • Balloon Enteroscopy: Balloon-assisted enteroscopy allows for direct visualization of the small intestine using an endoscope with an inflatable balloon at its tip. 
  • Capsule Endoscopy: Capsule endoscopy involves swallowing a small, wireless camera capsule that captures images of the gastrointestinal tract as it passes through the digestive system. 

Capsule endoscopy is particularly useful for evaluating the small intestine, which may be difficult to visualize with traditional endoscopy. 

  • Initial Assessment and Diagnosis: The first phase involves a comprehensive evaluation to assess the patient’s clinical presentation, medical history, symptoms, and laboratory findings. 
  • Stabilization and Symptom Management: Once a diagnosis of PLE is established, the focus shifts to stabilizing the patient and managing symptoms. 
  • Monitoring and Follow-Up: Ongoing monitoring and follow-up are essential components of PLE management to assess treatment response, monitor disease progression, and adjust therapy as needed. 
  • Long-Term Management and Support: The final phase of PLE management involves long-term monitoring, maintenance therapy, and support to manage the chronic nature of the condition. 

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