Eosinophilic gastroenteritis
| Eosinophilic gastroenteritis | |
 | |
|---|---|
| H&E Stain: Dense Eosinophilic infiltration of gastro-duodenal wall | |
| ICD-10 | K52.8 |
| ICD-9 | 558.3 |
| DiseasesDB | 32555 |
| eMedicine | med/688 |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Fatimo Biobaku M.B.B.S [2]
Overview
Eosinophilic gastroenteritis (EG) is a rare disorder characterized by localized patchy or diffuse eosinophilic infiltration of the gastrointestinal (GI) tissue. The presentation may vary depending on the location, depth and extent of bowel wall involvement. It usually runs a chronic relapsing course.[1][2][3][4]
Historical Perspective
It was first described by Kaijser in 1937.[5][6]
Classification
Eosinophilic gastroenteritis can be subdivided into three groups according to the Klein classification:[7][6][8]
- Mucosal
- Muscular
- Subserosal
Pathophysiology
The damage to the gastrointestinal tract wall is caused by eosinophilic infiltration and degranulation.[9] As a part of host defense mechanism, eosinophil is normally present in gastrointestinal mucosa, though finding in deeper tissue is almost always pathologic.[10] 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.[11] 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.[12] 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.[13]
Causes
The cause of eosinophilic gastroenteritis is unknown.[6]
Differentiating Eosinophilic gastroenteritis from Other Diseases
Epidemiology and Demographics
Incidence
The incidence rate differs between studies, as eosinophilic gastroenteritis is a rare disease.[6] The number of reported cases of EG are small, with approximately 300 EG cases reported in published literature. Serous membrane|Subserosal]] EG has an incidence rate of 4.5 % to 9 % in Japan, and 13 % in the USA.[14]
Age
EG can present at any age.[6] Earlier studies showed a higher incidence in the third to fifth decade of life.[15][1]
Sex
There is a slightly higher incidence in males.[16]
Race
Eosinophilic gastroenteritis has been documented in all races.[17]
Risk Factors
Screening
Natural History, Complications, and Prognosis
Natural History
Complications
Prognosis
Diagnosis
Diagnostic Criteria
Talley et al.[7] suggested 3 diagnostic criteria which is still widely used:
- the presence of gastrointestinal symptoms,
- 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),
- 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.[18] Sometimes diffuse inflammation results in complete loss of villi, involvement of multiple layers, submucosal oedema and fibrosis.[19][20]
Definitive diagnosis involves histological evidence of eosinophilic infiltration in biopsy slides. Microscopy reveals >20 eosinophils per high power field.[7][6] Infiltration is often patchy , can be missed and laparoscopic full thickness biopsy may be required.
Radio isotope scan using Tc-99m HMPAO-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.[21][22]
When eosinophilic gastroenteritis is observed in association with eosinophilic infiltration of other organ systems, the diagnosis of idiopathic hypereosinophilic syndrome should be considered.[23]
History and Symptoms
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.[24]; a high degree of clinical suspicion is often required to establish the diagnosis, as the disease is extremely rare. Occasionally, the disease may manifest itself as an acute abdomen or bowel obstruction.[25][26]
- Mucosal EG (25-100%) is the commonest variety.[6][27] It 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%) presents 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).[28] presents with ascites which is usually exudative in nature, abundant peripheral eosinophilia, and has favourable responses to corticosteroids.
- Other documented features are Cholangitis, pancreatitis,[29] eosinophilic splenitis, acute appendicitis and giant refractory duodenal ulcer.
Physical Examination
Laboratory Findings
Peripheral blood eosinophilia and elevated serum IgE are often seen
Imaging Findings
Other Diagnostic Studies
Treatment
Medical Therapy
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.[30] An elimination diet may be successful if a limited number of food allergies are identified.[31][18]
Surgery
Prevention
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
Allergy
Gastroenteritis
Malabsorption
References
- ↑ 1.0 1.1 Klein NC, Hargrove RL, Sleisenger MH, Jeffries GH (1970). "Eosinophilic gastroenteritis". Medicine (Baltimore). 49 (4): 299–319. PMID 5426746.
- ↑ Treiber GG, Weidner S (2007). "Eosinophilic gastroenteritis". Clin Gastroenterol Hepatol. 5 (5): e16. doi:10.1016/j.cgh.2007.01.011. PMID 17428742.
- ↑ Christopher V, Thompson MH, Hughes S (2002). "Eosinophilic gastroenteritis mimicking pancreatic cancer". Postgrad Med J. 78 (922): 498–9. PMC 1742453. PMID 12185230.
- ↑ Jimenez-Saenz M, Villar-Rodriguez JL, Torres Y, Carmona I, Salas-Herrero E, Gonzalez-Vilches J; et al. (2003). "Biliary tract disease: a rare manifestation of eosinophilic gastroenteritis". Dig Dis Sci. 48 (3): 624–7. PMID 12757181.
- ↑ Whitaker IS, Gulati A, McDaid JO, Bugajska-Carr U, Arends MJ (2004). "Eosinophilic gastroenteritis presenting as obstructive jaundice". Eur J Gastroenterol Hepatol. 16 (4): 407–9. PMID 15028974.
- ↑ 6.0 6.1 6.2 6.3 6.4 6.5 6.6 Whitaker IS, Gulati A, McDaid JO, Bugajska-Carr U, Arends MJ (2004). "Eosinophilic gastroenteritis presenting as obstructive jaundice". Eur J Gastroenterol Hepatol. 16 (4): 407–9. PMID 15028974.
- ↑ 7.0 7.1 7.2 Talley NJ, Shorter RG, Phillips SF, Zinsmeister AR (1990). "Eosinophilic gastroenteritis: a clinicopathological study of patients with disease of the mucosa, muscle layer, and subserosal tissues". Gut. 31 (1): 54–8. PMC 1378340. PMID 2318432 2318432 Check
|pmid=value (help). - ↑ Lee CM, Changchien CS, Chen PC, Lin DY, Sheen IS, Wang CS; et al. (1993). "Eosinophilic gastroenteritis: 10 years experience". Am J Gastroenterol. 88 (1): 70–4. PMID 8420276.
- ↑ Tan AC, Kruimel JW, Naber TH (2001). "Eosinophilic gastroenteritis treated with non-enteric-coated budesonide tablets". Eur J Gastroenterol Hepatol. 13 (4): 425–7. PMID 11338074 11338074 Check
|pmid=value (help). - ↑ Blackshaw AJ, Levison DA (1986). "Eosinophilic infiltrates of the gastrointestinal tract". J Clin Pathol. 39 (1): 1–7. PMC 499605. PMID 2869055 2869055 Check
|pmid=value (help). - ↑ Desreumaux P, Bloget F, Seguy D, Capron M, Cortot A, Colombel J, Janin A (1996). "Interleukin 3, granulocyte-macrophage colony-stimulating factor, and interleukin 5 in eosinophilic gastroenteritis". Gastroenterology. 110 (3): 768–74. PMID 8608886.
- ↑ Mishra A, Hogan S, Brandt E, Rothenberg M (2001). "An etiological role for aeroallergens and eosinophils in experimental esophagitis". J. Clin. Invest. 107 (1): 83–90. PMID 11134183.
- ↑ Pérez-Millán A, Martín-Lorente J, López-Morante A, Yuguero L, Sáez-Royuela F (1997). "Subserosal eosinophilic gastroenteritis treated efficaciously with sodium cromoglycate". Dig. Dis. Sci. 42 (2): 342–4. PMID 9052516.
- ↑ Miyamoto T, Shibata T, Matsuura S, Kagesawa M, Ishizawa Y, Tamiya K (1996). "Eosinophilic gastroenteritis with ileus and ascites". Intern. Med. 35 (10): 779–82. PMID 8933185.)
- ↑
- ↑ Guandalini, Stefano (2004). Essential Pediatric Gastroenterology and Nutrition. City: McGraw-Hill Professional. ISBN 0071416307. Page 210.
- ↑ Guandalini, Stefano (2004). Essential Pediatric Gastroenterology and Nutrition. City: McGraw-Hill Professional. ISBN 0071416307. Page 210.
- ↑ 18.0 18.1 Chen M, Chu C, Lin S, Shih S, Wang T (2003). "Eosinophilic gastroenteritis: clinical experience with 15 patients". World J. Gastroenterol. 9 (12): 2813–6. PMID 14669340.
- ↑ Johnstone J, Morson B (1978). "Eosinophilic gastroenteritis". Histopathology. 2 (5): 335–48. PMID 363591.
- ↑ Katz A, Goldman H, Grand R (1977). "Gastric mucosal biopsy in eosinophilic (allergic) gastroenteritis". Gastroenterology. 73 (4 Pt 1): 705–9. PMID 892374.
- ↑ Lee K, Hahm K, Kim Y, Kim J, Cho S, Jie H, Park C, Yim H (1997). "The usefulness of Tc-99m HMPAO labeled WBC SPECT in eosinophilic gastroenteritis". Clinical nuclear medicine. 22 (8): 536–41. PMID 9262899.
- ↑ Imai E, Kaminaga T, Kawasugi K, Yokokawa T, Furui S (2003). "The usefulness of 99mTc-hexamethylpropyleneamineoxime white blood cell scintigraphy in a patient with eosinophilic gastroenteritis". Annals of nuclear medicine. 17 (7): 601–3. PMID 14651361.
- ↑ Matsushita M, Hajiro K, Morita Y, Takakuwa H, Suzaki T (1995). "Eosinophilic gastroenteritis involving the entire digestive tract". Am. J. Gastroenterol. 90 (10): 1868–70. PMID 7572911.
- ↑ Christopher V, Thompson M, Hughes S (2002). "Eosinophilic gastroenteritis mimicking pancreatic cancer". Postgraduate medical journal. 78 (922): 498–9. PMID 12185230.
- ↑ Shweiki E, West J, Klena J, Kelley S, Colley A, Bross R, Tyler W (1999). "Eosinophilic gastroenteritis presenting as an obstructing cecal mass--a case report and review of the literature". Am. J. Gastroenterol. 94 (12): 3644–5. PMID 10606337.
- ↑ Tran D, Salloum L, Tshibaka C, Moser R (2000). "Eosinophilic gastroenteritis mimicking acute appendicitis". The American surgeon. 66 (10): 990–2. PMID 11261632.
- ↑ Lee C, Changchien C, Chen P, Lin D, Sheen I, Wang C, Tai D, Sheen-Chen S, Chen W, Wu C (1993). "Eosinophilic gastroenteritis: 10 years experience". Am. J. Gastroenterol. 88 (1): 70–4. PMID 8420276.
- ↑ Miyamoto T, Shibata T, Matsuura S, Kagesawa M, Ishizawa Y, Tamiya K (1996). "Eosinophilic gastroenteritis with ileus and ascites". Intern. Med. 35 (10): 779–82. PMID 8933185.)
- ↑ Lyngbaek S, Adamsen S, Aru A, Bergenfeldt M (2006). "Recurrent acute pancreatitis due to eosinophilic gastroenteritis. Case report and literature review". JOP. 7 (2): 211–7. PMID 16525206.
- ↑ Barbie D, Mangi A, Lauwers G (2004). "Eosinophilic gastroenteritis associated with systemic lupus erythematosus". J. Clin. Gastroenterol. 38 (10): 883–6. PMID 15492606.
- ↑ Katz A, Twarog F, Zeiger R, Falchuk Z (1984). "Milk-sensitive and eosinophilic gastroenteropathy: similar clinical features with contrasting mechanisms and clinical course". J. Allergy Clin. Immunol. 74 (1): 72–8. PMID 6547462.