eosinophilic gastroenteritis

好酸球性胃腸炎

WordNet

1. of or relating to eosinophil
2. inflammation of the stomach and intestines; can be caused by Salmonella enteritidis (同)stomach flu, intestinal flu
3. a leukocyte readily stained with eosin (同)eosinophile

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1. 胃腸炎

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2015/06/16 20:31:25」(JST)

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Eosinophilic gastroenteritis
H&E Stain: Dense Eosinophilic infiltration of gastro-duodenal wall
Classification and external resources
ICD-10	K52.8
ICD-9	558.3
DiseasesDB	32555
eMedicine	med/688

Eosinophilic gastroenteritis (EG) is a rare and heterogeneous condition characterized by patchy or diffuse eosinophilic infiltration of gastrointestinal (GI) tissue, first described by Kaijser in 1937 . Presentation may vary depending on location as well as depth and extent of bowel wall involvement and usually runs a chronic relapsing course. It can be classified into mucosal, muscular and serosal types based on the depth of involvement. Any part of the GI tract can be affected, and isolated biliary tract involvement has also been reported. The stomach is the organ most commonly affected, followed by the small intestine and the colon .

Pathophysiology

Peripheral blood eosinophilia and elevated serum IgE are usual but not universal. The damage to the gastrointestinal tract wall is caused by eosinophilic infiltration and degranulation.

As a part of host defense mechanism, eosinophils are normally present in gastrointestinal mucosa, though the finding in deeper tissue is almost always pathologic . What triggers such dense infiltration in EG is not clear. It is possible that different pathogenetic mechanisms of disease is involved in several subgroups of patients. Food allergy and variable IgE response to food substances has been observed in some patients which implies role of hypersensitive response in pathogenesis. Many patients indeed have history of other atopic conditions like eczema, asthma etc.

Eosinophil recruitment into inflammatory tissue is a complex process, regulated by a number of inflammatory cytokines. In EG cytokines IL-3, IL-5 and granulocyte macrophage colony stimulating factor (GM-CSF) may be behind the recruitement and activation. They have been observed immunohistochemically in diseased intestinal wall . In addition eotaxin has been shown to have an integral role in regulating the homing of eosinophils into the lamina propria of stomach and small intestine . In the allergic subtype of disease, it is thought that food allergens cross the intestinal mucosa and trigger an inflammatory response that includes mast cell degranulation and recruitment of eosinophils.

Symptoms and signs

EG typically presents with a combination of chronic nonspecific GI symptoms which include abdominal pain, nausea, vomiting, diarrhea, weight loss, and abdominal distension. Approximately 80% have symptoms for several years; a high degree of clinical suspicion is often required to establish the diagnosis, as the disease is extremely rare. It doesn't come all of a sudden but takes about 3-4 years to develop depending upon the age of the patient. Occasionally, the disease may manifest itself as an acute abdomen or bowel obstruction.

• Mucosal EG (25-100%) is the most common variety, which presents with features of malabsorption and protein losing enteropathy. Failure to thrive and anaemia may also be present. Lower gastrointestinal bleeding may imply colonic involvement.
• Muscular EG (13-70%) present with obstruction of gastric outlet or small intestine; sometimes as an obstructing caecal mass or intussusception.
• Subserosal EG (4.5% to 9% in Japan and 13% in the USA) presents with ascites which is usually exudative in nature, abundant peripheral eosinophilia, and has favourable responses to corticosteroids.
• Other documented features are Cholangitis, pancreatitis, eosinophilic splenitis, acute appendicitis and giant refractory duodenal ulcer.

Diagnosis

Spiral CT showing ascites and concentric thickening of colon and ileum in EG

Talley et al. suggested 3 diagnostic criteria which is still widely used:

1. the presence of gastrointestinal symptoms,
2. histological demonstration of eosinophilic infiltration in one or more areas of the gastrointestinal tract or presence of high eosinophil count in ascitic fluid (latter usually indicates subserosal variety),
3. no evidence of parasitic or extraintestinal disease.

Hypereosinophilia, the hallmark of allergic response, may be absent in up to 20% of patients, but hypoalbuminaemia and other abnormalities suggestive of malabsorption may be present.

CT scan may show nodular and irregular thickening of the folds in the distal stomach and proximal small bowel, but these findings can also be present in other conditions like Crohn's disease and lymphoma.

The endoscopic appearance in eosinophilic gastroenteritis is nonspecific; it includes erythematous, friable, nodular, and occasional ulcerative changes. Sometimes diffuse inflammation results in complete loss of villi, involvement of multiple layers, submucosal oedema and fibrosis.

Definitive diagnosis involves histological evidence of eosinophilic infiltration in biopsy slides. Microscopy reveals >20 eosinophils per high power field. Infiltration is often patchy, can be missed and laparoscopic full thickness biopsy may be required.

Radio isotope scan using technetium (^99mTc) exametazime-labeled leukocyte SPECT may be useful in assessing the extent of disease and response to treatment but has little value in diagnosis, as the scan does not help differentiating EG from other causes of inflammation.

When eosinophilic gastroenteritis is observed in association with eosinophilic infiltration of other organ systems, the diagnosis of idiopathic hypereosinophilic syndrome should be considered.

Management

Corticosteroids are the mainstay of therapy with a 90% response rate in some studies. Appropriate duration of steroid treatment is unknown and relapse often necessitates long term treatment. Various steroid sparing agents e.g. sodium cromoglycate (a stabilizer of mast cell membranes), ketotifen (an antihistamine), and montelukast (a selective, competitive leukotriene receptor antagonist) have been proposed, centering around an allergic hypothesis, with mixed results. An elimination diet may be successful if a limited number of food allergies are identified.

Epidemiology

Epidemiology may differ between studies, as number of cases are small, with approximately 300 EG cases reported in published literature.

EG can present at any age and across all races, with a slightly higher incidence in males. Earlier studies showed higher incidence in the third to fifth decades of life.

Other gastrointestinal conditions associated with allergy

• Eosinophilic esophagitis
• Eosinophilic ascites
• Coeliac disease
• Protein losing enteropathy from intolerance to cow's milk protein
• Infantile formula protein intolerance

See also

• Aeroallergen
• Allergy
• Gastroenteritis
• Malabsorption

External links

• Campaign Urging Research for Eosinophilic Disease
• American Partnership for Eosinophilic Disorders
• Families Affected By Eosinophilic Disorders
• Cincinnati Center for Eosinophilic Disorders
• Hoover boy's rare disease leaves him allergic to almost everything he eats
• Canadian Council of Eosinophilic Disorders

References

UpToDate Contents

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• 1. 好酸球性胃腸炎 eosinophilic gastroenteritis
• 2. 好酸球の生物学および好酸球増多症の原因 eosinophil biology and causes of eosinophilia
• 3. 好酸球性食道炎の臨床症状および診断 clinical manifestations and diagnosis of eosinophilic esophagitis
• 4. 好酸球性食道炎の治療 treatment of eosinophilic esophagitis
• 5. 好酸球性肉芽腫性多発血管炎(チャーグ・ストラウス症候群）の臨床的特徴および診断 clinical features and diagnosis of eosinophilic granulomatosis with polyangiitis churg strauss

English Journal

• Severe odynophagia and rapidly progressive Dysphagia as an unusual presentation of eosinophilic gastroenteritis.

• Rodrigues CG, Oliveira A, Deus JR.
• Clinical gastroenterology and hepatology : the official clinical practice journal of the American Gastroenterological Association.Clin Gastroenterol Hepatol.2014 Apr;12(4):e27-8. doi: 10.1016/j.cgh.2013.08.013. Epub 2013 Aug 17.
• PMID 23962546

• Eosinophilic ascites with marked peripheral eosinophilia: a diagnostic challenge.

• Elliott JA1, McCormack O, Tchrakian N, Conlon N, Ryan CE, Lim KT, Ullah N, Mahmud N, Ravi N, McKiernan S, Feighery C, Reynolds JV.Author information 1aDepartment of Surgery, Trinity Centre, St James's Hospital and Trinity College bDepartment of Pathology cDepartment of Immunology dDepartment of Gastroenterology, St James's Hospital, Dublin, Ireland.AbstractEosinophilic disease of the gastrointestinal tract is rare and is characterized by the presence of gastrointestinal symptoms in association with eosinophilic infiltration of any part of the gastrointestinal tract. Clinical presentation of eosinophilic gastroenteritis (EGE) varies not only by the part of the gastrointestinal tract involved but also with the depth of eosinophilic infiltration of the gut wall. We describe the case of a 41-year-old woman with a history of atopy who presented with severe abdominal pain and diarrhoea. Investigations showed large-volume eosinophil-rich ascites and a markedly elevated peripheral blood eosinophil count and immunoglobulin E level. Bone marrow aspirate, trephine biopsy and T-cell studies showed no evidence of underlying haematological malignancy. Vasculitic disease and parasitic infection were systematically excluded. Colonic and upper gastrointestinal biopsies confirmed a diagnosis of EGE with eosinophilic ascites. The patient was treated with systemic corticosteroids and dietary allergen elimination with dramatic therapeutic response. The diagnostic and therapeutic challenges associated with EGE in its various forms are discussed.
• European journal of gastroenterology & hepatology.Eur J Gastroenterol Hepatol.2014 Apr;26(4):478-84. doi: 10.1097/MEG.0000000000000037.
• Eosinophilic disease of the gastrointestinal tract is rare and is characterized by the presence of gastrointestinal symptoms in association with eosinophilic infiltration of any part of the gastrointestinal tract. Clinical presentation of eosinophilic gastroenteritis (EGE) varies not only by the par
• PMID 24535594

• Eosinophilic Gastroenteritis Cured with Helicobacter pylori Eradication: Case Report and Review of Literature.

• Soavi C1, Caselli M, Sioulis F, Cassol F, Lanza G, Zuliani G.
• Helicobacter.Helicobacter.2014 Mar 21. doi: 10.1111/hel.12122. [Epub ahead of print]
• PMID 24650300

Japanese Journal

• 鶏卵アレルギーに対する急速経口免疫療法によって発症したと考えられた好酸球性食道-胃腸炎の1例

• アレルギー 64(1), 57-62, 2015-02-01
• NAID 110009908914

• Proton pump inhibitor treatment decreased duodenal and esophageal eosinophilia in a case of eosinophilic gastroenteritis

• Allergology International 64(Supplement.1), 83-85, 2015
• NAID 130005099478

• 鶏卵アレルギーに対する急速経口免疫療法によって発症したと考えられた好酸球性食道―胃腸炎の1例

• アレルギー 64(1), 57-62, 2015
• NAID 130005091001

Related Links

• Eosinophilic Gastroenteritis: Treatment of EGE

What is the etiology of eosinophilic gastroenteritis and what are the most promising treatment options for this gastrointestinal disease? ... Table 1. Summary of eosinophilic gastroenteritis symptoms and common findings, according ...

• Eosinophilic Gastroenteritis

Muscular form: appearing in 12–30% of cases. [3,13] In these patients, the inflammation extends deeper into the muscle layers, leading to digestive wall thickening and typical obstructive symptoms. Although any section of the digestive tract can be involved, the stomach and duodenum are the ...

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★リンクテーブル★

リンク元	「好酸球性胃腸炎」
関連記事	「eosinophilic」

「好酸球性胃腸炎」

　　[★]

英

eosinophilic gastroenteritis

「eosinophilic」

　　[★]

• adj.

• 好酸球性の、好酸球の、好酸性の、好酸の、エオシン好性の、エオジン好性の

関

acidophil、acidophile、acidophilic、eosinophil