Eosinophilic gastroenteritis, also known as eosinophilic gastroenteritis, is a rare disease characterized by diffuse or limited eosinophilic infiltration in certain parts of the gastrointestinal tract. Clinical manifestations include crampy epigastric pain, which may be accompanied by nausea, vomiting, fever, or a history of specific food allergies. The disease was first reported by Kaizser in 1937, and to date about 200 cases have been reported in the world literature.
Disease Overview
Eosinophilic gastroenteritis is a disease of the gastrointestinal tract characterized by an increase in peripheral blood eosinophils. It can occur at any age, with the most frequent occurrence between 20 and 50 years of age. There are no gender differences.
The clinical signs and symptoms of eosinophilic gastroenteritis depend on the depth of infiltration of the gastrointestinal wall and the presence or absence of esophagogastric and colonic lesions, and can be clinically classified into 3 types. Mucosal type (Type I) Common symptoms include nausea, vomiting, abdominal pain, diarrhea, weight loss, and low back pain, which can be aggravated by eating specific allergic foods. Physical examination may reveal skin eczema, urticaria, and foot and ankle edema. Some patients present with anemia. Myxomatous type (Type II) The main manifestations are complete or incomplete pyloric and small bowel obstruction, nausea, vomiting, and abdominal pain, which are difficult to be relieved by antacids or anticholinergics. The plasma membrane type (type III) is relatively rare and accounts for about 10% of the total eosinophilic gastroenteritis. Ascites may occur.
Disease classification
Gastroenterology
Disease description
Eosinophilic gastroenteritis is a gastrointestinal disease characterized by an increase in peripheral blood eosinophils, with varying degrees of eosinophil infiltration in the stomach and small intestine. The etiology is unclear and is associated with allergic reactions and immune dysfunction. It responds well to glucocorticosteroid therapy and is more common in young adults and less common in children.
Symptoms and signs
Eosinophilic gastroenteritis can occur at any age, with the most frequent occurrence between 20 and 50 years of age. There are no gender differences. The lesions may invade the entire mucosa of the gastrointestinal tract from the esophagus to the colon. The clinical signs and symptoms of eosinophilic gastroenteritis depend on the depth of infiltration of the gastrointestinal wall and the presence or absence of esophagogastric and colonic lesions, and can be clinically classified into 3 types.
1, mucosal type (type I) lesions mainly invade the gastrointestinal mucosa tissue, common symptoms are nausea, vomiting, abdominal pain, diarrhea, weight loss and low back pain, eating special allergic food can make the symptoms worse. Physical examination may reveal skin eczema, urticaria, and foot and ankle edema. Some patients may also present with anemia due to gastrointestinal bleeding. When the lesions are extensive, systemic manifestations such as small intestine malabsorption, protein-losing enteropathy, anemia, etc. may occur. Adolescents may develop dysplasia and growth retardation, and women may have secondary amenorrhea.
2. Myenteric type (Type II) Myenteric lesion is the main lesion. Due to the thickening and stiffness of the stomach and small intestine wall, the main clinical manifestations are complete or incomplete pyloric and small intestine obstruction, nausea, vomiting and abdominal pain, which are difficult to be relieved by antacids or anticholinergics.
3, plasma membrane type (type III) Subplasma membrane lesions are predominant, relatively rare, accounting for about 10% of the entire eosinophilic gastroenteritis. Ascites may appear, and the ascites contains a large number of eosinophils. This type may exist alone or in combination with the other two types.
Disease etiology
The etiology of eosinophilic gastroenteritis is unclear. Food allergy is assumed, but it is common in children, and adults rarely find allergic foods.
Pathophysiology
Eosinophilic gastroenteritis is characterized by eosinophil infiltration in the tissues. It is believed that the disease is stimulated by certain substances causing a systemic or local allergic reaction, a syndrome secondary to some unexplained antigen and allergic reaction. It is generally believed that the disease develops through the degranulation of eosinophils and the release of various enzymes that cause tissue damage. Based on the fact that the disease is effectively treated with hormones, that some patients have increased blood IgE, and that there is a history of allergy or family history, the type I allergic reaction hypothesis is proposed. It is believed that the integrity of the gastrointestinal mucosa is damaged by certain factors, and antigens such as food enter the tissue, causing mast cells to sensitize and degranulate, releasing histamine and eosinophil chemokines, causing eosinophil infiltration and degranulation, resulting in tissue damage, and the released eosinophil peroxidase further stimulates mast cells to release histamine, forming a vicious circle. In addition to extranodal type I allergic reactions, abnormal immune function plays an important role in the pathogenesis of eosinophilic gastroenteritis, but no consistent abnormal immune function has been found. It has been proposed that eosinophilic gastroenteritis is a specific inflammation of the GI tract and that this eosinophilic inflammation is an allergic reaction caused by certain unidentified antigens that can be part of the reaction to Crohn’s disease, ulcerative colitis, amoebic dysentery, and lacto-protein colitis. There are reports of a genetic predisposition to eosinophilic enteritis, which cannot be conclusive as there are no familial investigations. The site of involvement can range from the esophagus to all segments of the rectum, with the small intestine and stomach being the most commonly involved. Eosinophilic infiltration may involve the entire gastrointestinal wall or may be predominant in one layer. The most common layer is the mucosa and submucosa, followed by the muscularis, and the plasma layer is rare. Other pathological changes include edema, small intestinal villous atrophy, necrosis and regeneration of mucosal and glandular epithelial cells. Eosinophil infiltration is seen in any inflammatory process and should be differentiated when there is a significant increase in other inflammatory cells.
Diagnostic tests
Diagnosis: The diagnosis is mainly based on clinical manifestations, blood picture, radiological and endoscopic plus pathological findings.
1.Talley criteria
(1) The presence of gastrointestinal symptoms.
(2) Biopsy pathology shows eosinophil infiltration in one or more parts of the gastrointestinal tract from the esophagus to the colon, or radiological colonic abnormalities with peripheral eosinophilia.
(3) Excluding parasitic infections and diseases with eosinophilia outside the gastrointestinal tract, such as connective tissue disease, eosinophilia, Crohn’s disease, lymphoma, primary amyloidosis Menetrieri disease, etc.
2.Leinbach’s criteria
(1) Gastrointestinal symptoms and signs after eating specific foods.
(2) Eosinophilia in peripheral blood.
(3) Histological evidence of eosinophilia or infiltration of the gastrointestinal tract.
Laboratory tests.
1, blood tests 80% of patients have peripheral blood eosinophilia, mucosal and submucosal lesions and myelopathy dominant patients (1 to 2) × 109/L, plasma cell lesions dominant, up to 8 × 109/L. There may also be iron deficiency anemia, decreased serum albumin, increased blood IgE, increased sedimentation.
The significance of fecal examination in acid cell gastroenteritis is to exclude intestinal parasitic infections, some may see Charcot-Leyden crystals, positive occult blood in routine stool examination, some patients have mild to moderate steatorrhea, increased Cr marker albumin, increased clearance of alpha-antitrypsin, abnormal D-xylose absorption test.
Other ancillary tests.
1, X-ray examination Eosinophilic gastroenteritis lacks specificity. Barium X-ray meal reveals mucosal edema, widened folds, nodular filling defects, thickened gastrointestinal wall, luminal narrowing and obstruction.
2.CT examination can find thickening of the gastrointestinal wall, enlarged mesenteric lymph nodes or ascites.
3.Endoscopy and biopsy For eosinophilic gastroenteritis with predominantly mucosal and submucosal lesions. Microscopically, coarse mucosal folds, congestion, edema, ulcers or nodules are seen, and biopsy confirms pathologically a large number of eosinophilic infiltrates, which is valuable for confirming the diagnosis. However, biopsy tissue is of little value in patients with predominantly myxomatous and plasma layer involvement, and sometimes requires surgical pathological confirmation.
4, laparotomy patients with ascites must perform diagnostic laparotomy, ascites is exudative, containing a large number of eosinophils, must do staining of ascites smear to distinguish between eosinophils and neutrophils.
5.Laparoscopic examination Lack of specific manifestation under laparoscopy. In mild cases, there is only peritoneal congestion, and in severe cases, it may resemble peritoneal metastatic cancer. The significance of laparoscopy is to perform biopsy of peritoneal mucosal tissue and get pathological diagnosis.
6.Surgical exploration For suspected eosinophilic gastroenteritis usually not confirmed by dissection, but surgery is performed when there is intestinal obstruction or pyloric obstruction or suspected tumor.
Differential diagnosis
1, dyspepsia Patients with eosinophilic gastroenteritis may have symptoms of dyspepsia such as abdominal pain, nausea, vomiting, and abdominal distention, but often lack specificity. For patients with dyspepsia as a manifestation, they should be differentiated from peptic ulcer, reflux esophagitis, gastric cancer, chronic pancreatitis, etc.
2. Intestinal obstruction Intestinal obstruction can often occur in myxomatous type, and attention should be paid to exclude gastrointestinal tumors and intestinal vascular diseases, etc.
3. ascites Most often seen in plasma membrane type eosinophilic gastroenteritis. Routine and biochemical examination of ascites, CEA test of ascites and pathological examination of ascites can help to diagnose the disease.
4, eosinophilia is a systemic disease of unknown etiology, it can also involve the gastrointestinal tract. 60% involve the liver, 14% involve the gastrointestinal tract, diffuse eosinophilic gastroenteritis often involves 50% of the organs outside the gastrointestinal tract in addition to the gastrointestinal tract.
5. Intestinal parasitic infections can cause a variety of non-specific GI symptoms, along with peripheral blood eosinophilia. Repeated examination of fecal eggs can be identified.
6, allergic diseases Bronchial asthma, allergic rhinitis, urticaria, in addition to the peripheral blood eosinophilia, each have their own clinical manifestations.
7, eosinophilic granuloma mainly occurs in the stomach, large and small intestine, a limited mass, peripheral blood eosinophils are generally not elevated, the pathology is characterized by eosinophilic granuloma mixed in the connective tissue stroma.
8, rheumatic diseases Various vasculitis, such as allergic granulomatosis (Churg-Strauss syndrome) and polyarteritis nodosa, other connective tissue diseases such as scleroderma, dermatomyositis and polymyositis can involve the gastrointestinal tract, with symptoms such as abdominal pain and dyspepsia, which can present with varying degrees of peripheral blood eosinophilia. Small intestinal mucosal biopsy can help in the differential diagnosis.
Treatment plan
1.General treatment For identified or suspected foods, drugs should be stopped, some specific foods milk, eggs, meat fish and shrimp, etc., the symptoms can be reduced after controlled eating.
2, drug treatment Glucocorticosteroid treatment has good efficacy, prednisone 20-40mg per day, 1-2 weeks after taking the drug symptom control can be reduced to maintain, gradually stop the drug. Sodium cromoglycate can inhibit the degranulation of mast cells and prevent the release of histamine, 40-60mg per day, 3 times/d, the course of treatment varies from 6 weeks to several months, suitable for hormone therapy is ineffective.
3.Surgical treatment When pyloric obstruction and intestinal obstruction occur, if the conservative medical treatment is ineffective, surgical treatment can be adopted, which is prone to recurrence and requires hormone maintenance treatment after surgery.
Prevention prognosis
Prognosis: The prognosis of this disease is good, and no malignant changes have been reported.
Prevention: No relevant information is available.
Complications
Adolescent onset may lead to growth retardation and amenorrhea. The typical clinical manifestation when myelomeningocele is predominantly involved is intestinal obstruction or pyloric obstruction with corresponding signs and symptoms. Occasionally, eosinophilic infiltration of the esophageal muscular layer causes cardia incontinentia. Plasma layer involvement is the least common and typically presents as ascites, with large numbers of eosinophils seen in the ascites.
Epidemiology
The disease is rare and was first reported by Kaijser in 1937, with less than 300 cases in the literature to date. It occurs mainly in young people between 20 and 30 years of age, but can also occur in children and the elderly; the incidence in men is approximately twice that in women; its population incidence is difficult to determine, with limited data suggesting only 1 case of EG per 100,000 hospitalizations.
What are the diseases that can be confused with it?
The following diseases should be differentiated from this disease.
(1) Parasitic infections Peripheral blood eosinophilia can be seen in parasitic diseases caused by hookworms, schistosomes, cestodes, and cystic nematodes, each with its own clinical manifestations.
(2) Gastrointestinal tract carcinoma and malignant lymphoma may also have peripheral blood eosinophilia, but it is secondary to other manifestations of carcinoma and lymphoma.
(3) Eosinophilic granuloma mainly occurs in the stomach and small intestine as a limited mass, and the histological examination is eosinophilic granuloma mixed in the connective tissue matrix.
(4) Eosinophilia In addition to increased peripheral blood eosinophils, the lesion not only involves the intestine but also extensively involves other parenchymal organs such as the brain, heart, lungs and kidneys, etc. The short course of the disease has a poor prognosis and death often occurs within a short period of time.