Occurs when the intestinal mucosa fails to function as a barrier separating the body from the outside world.
The two main pathological mechanisms that compromise epithelial retention of interstitial, contents are direct damage to the mucosa and failed lymph drainage, causing backflow through the epithelium into the lumen.
Mucosal epithelial permeability occurs with inflammation, infection, allergic reactions, or a genetic abnormality altering the mucosa.
Some diseases involve other mechanisms, in addition to the above
PLE, is an uncompensated loss of plasma proteins in the intestine, indicated by elevated alpha 1 antitrypsin levels in the stool, which leads to panhypoproteinemia in the absence of liver or kidney disease.
Results in the leakage of serum fluids and proteins into the stool with diarrhea, and hypoalbuminemia..
May be related to loss of heparin sulfate proteins normally expressed on intestinal cells.
Refers to any condition of the gastrointestinal tract that results in a net loss of protein from the body.
A local vasculitis, or inflammatory, cytokines, secretion can aggravate proteins, losing interrupt tea by increasing interstitial fluid accumulation.
PLE is manifested, as non-selective depletion of all plasma proteins includes albumin, immunoglobulins, and acute phase reactants.
Mucosal erosion, occurs in inflammatory bowel disease, peptic, ulcer, infections, and carcinoma and cause loss of plasma proteins into the gut lumen.
This is commonly seen in Crohn’s disease, ulcerative, colitis, erosive, gastritis, and pseudomembranous colitis.
Other inflammatory conditions with erosive, PLE include: sarcoidosis, graft versus host disease, G.I. amyloidosis.
PLE can occur in SLE, Sjogren syndrome, Henoch-Schonlein purpura, and pemphigus vulgaris.
PLE may be seen in Meneyriet’s disease, usually an nonerosive hypertrophic gastropathy involving overgrowth of mucus secreting cells, decreased gastric acid, and sometimes gastric cancer.
Juvenile polyposis syndrome can manifest as PLE.
Infections can precipitate inflammatory mucosal damage as well.
Uncommonly malignancies involving epithelial tumors of the esophagus, stomach and intestine and mucosal metastatic disease can lead to mucosal drainage and PLE.
Where really, PLE is seen in AIDS who have Kaposi’s sarcoma and patients with organ transplantation.
Allergic disorders, including eosinophilic gastroenteritis may cause PLE and food elimination diets converse the process.
Celiac disease is frequently associated with PLE, which correlates with the degree of villous architecture abnormality and responds to a gluten free diet.
Diseases that impair epithelial barrier, and allow loss of interstitial fluid proteins into the intestinal contents can result in PLE.
Heparin sulfate proteoglycans help maintain tight intracellular functions, and PLE can occur when intestinal epithelial cellslack heparin sulfate.
A selective protein deficiency indicates conditions, other than PLE: hypoalbuminemia with normal immunoglobulin levels may reflect liver disease, or reduced low molecular weight proteins, indicate the nephrotic syndrome, or other kidney diseases, and low immunoglobulin level suggest immunodeficiency.
PLE, involves the wasting of all other intestinal fluid components.
The pressure of each heartbeat drives fluid with albumin, immunoglobulins, and other serum components from peripheral blood vessels into the interstitium.
Inflammation permits capillary permeability, and interstitial influx.
Interstitial fluid is recirculated by the lymphatics, which are tributaries to the arterial venous circulation that start as opened vessels in the interstitium.
Lymphatics have valves for one-way flow down the shallow pressure gradient to the thoracic duct, which returns to the subclavian vein. Lymph recirculation requires an intact vasculature with a pressure gradient guiding unidirectional flow to the right side of the heart.
Cardiac conditions causing chronically elevated central venous pressure impede lymph return from intestinal lymphatics: constrictive, pericarditis, long-standing CHF can cause secondary intestinal lymphangiectasia.
Diseases block lymphatic drainage cause pathological distention, distortion, and disruption of lymphatics in a process, called intestinal lymphangiectasia, which massively causes protein loss into the bowel lumen, and a severe protein losing enteropathy.
Lymph flow is screened for pathogenic agents through lymph nodes.
Widespread or metastatic abdominal malignancies can block them for drainage and cause a PLE.
PLE can be a cause and consequence of thrombotic disease.
PLE promotes thrombosis in diseases, such as SLE, with or without phospholipid syndrome, Crohn’s disease, ulcerative colitis Waldenström’s macroglobulinemia.
Prothrombotic mechanisms include loss of inhibitory coagulation factors through gut wasting, or overproduction of the prothrombotic factor fibrinogen in the liver.
PLE, may be a consequence of blood clots thst large in the gut, especially in the mesenteric veins, and superior vena cava, which occur in primary thrombophilia conditions.
Lymphangiectasia can result from lymphatic damage or inflammation from infection with bacteria, viruses, fungi, or parasites.
Whipple’s disease due to Tropheryma whipplei a systemic chronic infection characterized by chronic diarrhea, hypoproteinemia, abdominal pain, malnutrition, and weight loss due to PLE, as well as migratory, polyarthralgia and enlarged lymph nodes.
Food, stuffs, nutrients, and micro nutrients, such as calcium, magnesium, zinc, iron, and copper, or absorbed in the gut interstitium of the villus, which contains a central intestinal, lymphatic or lacteal.
Other blood constituents, including hematopoietic cells, fats, and vitamins can also enter the lymph.
Epithelial cells connected by tight junctions guard the integrity and composition of the gut interstitium.
Proteins losing enteropathy occurs when the intestinal epithelium is breached and the intestitial fluid leaks into the gut lumen.
Interstitial fluid is recirculated by lymphatics, which are tributaries to the arterial venous circulation.
PLE involves the wasting of interstitial fluid compartments.
Each heartbeat drives fluid, containing albumin, immunoglobulins, and other serum components from peripheral blood vessels into the interstitium.
The signs/symptoms are consistent with diarrhea, fever, and general abdominal discomfort.
Presenting features of PLE include: hypoproteinemia, edema, nutritional deficiencies, infections, G.I. symptoms of diarrhea, steatorrhea, abdominal pain, and vomiting.
Hypoproteinemia is nonselective with decreased albumin and immunoglobulin levels.
Facial edema, and edema of the arms and legs, and effusions of the peritoneum, pleura and pericardium caused by reduced oncotic pressure in the blood.
Infections can result from hypogammaglobulinemia and lymphopenia.
Opportunistic infections, including cryptosporidium, cytomegalovirus, mycobacterium avium complex, may cause mesenteric and retroperotoneal obstruction of lymphatics and cause of protein losing enteropathy.
In children, malabsorption and malnutrition may be severe, resulting in retarded growth and development.
Swelling of the legs can occur.
The causes of protein-losing enteropathy include GI conditions:
Inflammatory bowel disease.
Idiopathic ulcerative jejunoileitis.
Infection
Neoplasm
Sarcoidosis
Amyloidosis
Systemic lupus erythematosus (SLE)
Ménétrier’s disease.
Zollinger-Ellison syndrome
Eosinophilic gastroenteritis
Celiac disease
Common variable immunodeficiency
Primary intestinal lymphangectasia
The process is a result of plasma proteins loss, which enters GI tract.
It is a complication of a disorder, be it lymphatic obstruction or mucosal disease.
DIAGNOSIS:
Diagnosis is made by excluding other causes of protein loss, and endoscopy can be used to localize its cause.
Diagnosis is by ruling out of the causes of hypoproteinemia and diarrhea, or malabsorption, and documenting enteric protein loss, performing imaging studies of the cardiovascular, thoracic, and abdominal pelvic structures, assessing systemic, manifestations of protein losing entteropathy, and providing pathological, and possibly genetic testing.
Endoscopy and biopsy can reveal malformations, obstruction, mucosal inflammation, ulcers, dilated lacteals, and lymph ataxia, and neoplasms
Fecal excretion of alpha 1-antitrypsin is a marker of protein losing enteropathy,
Gastrointestinal protein loss is assessed by measuring fecal alpha1-antitrypsin, or more accurately, by measuring alpha1- antitrypsin clearance in a 24 hour school collection with simultaneous serum measurement.
Hypoproteinemia is the most frequent manifestation of PLE: ruling out disorders of reduce protein synthesis, such as with liver disease, or malnutrition, increased, urinary excretion of proteins, increased protein, catabolism, and altered tissue distribution of proteins with increased capillary permeability.
Treatment depends upon the underlying process.
Therapies include fluid and electrolyte abnormality, corrections, nutritional management, replacing trace elements, prevention or control of target organ complications.
Controlling peripheral edema, promoting ambulation to avoid venous thrombosis, , antibiotics to control infections, skin care to prevent breakdown, or additional measures.
Albumin infusions may stabilize the clinical situation, but does not provide long lasting benefit.
Patients may require immunoglobulin replacement therapy, and anti-biotic prophylaxis, where there is recurrent infections and profound immunodeficiency.
Drugs that may be used to alleviate PLE, include octreotide, heparin analogues, anti-plasmon therapy, corticoids, sitolimus and everolimus.
High protein and lower fat diet maybe effective treatment.