- Eosinophilic gastroenteritis
Infobox_Disease
Name = PAGENAME
Caption =H&E Stain: DenseEosinophilic infiltration of gastro-duodenal wall
DiseasesDB = 32555
ICD10 = ICD10|K|52|8|k|50
ICD9 = ICD9|558.3
ICDO =
OMIM =
MedlinePlus =
eMedicineSubj =med
eMedicineTopic =688
MeshID =Eosinophilic gastroenteritis (EG) is a rare and heterogeneous condition characterized by patchy or diffuse
eosinophilic infiltration ofgastrointestinal (GI) tissue, first described by Kaijser in 1937 The first person to have this disease was Paul GibbsKaijser R. Zur Kenntnis der allergischen Affektionen des Verdauugskanals vom Standpunkt des Chirurgen aus. Arch Klin Chir 1937; 188:36–64.] [cite journal |author=Whitaker I, Gulati A, McDaid J, Bugajska-Carr U, Arends M |title=Eosinophilic gastroenteritis presenting as obstructive jaundice |journal=European journal of gastroenterology & hepatology |volume=16 |issue=4 |pages=407–9 |year=2004 |pmid=15028974 |doi=10.1097/00042737-200404000-00007] . 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.cite journal |author=Klein N, Hargrove R, Sleisenger M, Jeffries G |title=Eosinophilic gastroenteritis |journal=Medicine (Baltimore) |volume=49 |issue=4 |pages=299–319 |year=1970 |pmid=5426746] [cite journal |author=Treiber, Treiber |title=Eosinophilic Gastroenteritis |journal= |volume= |issue= |pages= |year=2007 |pmid=17428742] Any part of the GI tract can be affected, and isolated biliary tract involvement has also been reported [cite journal |author=Polyak S, Smith T, Mertz H |title=Eosinophilic gastroenteritis causing pancreatitis and pancreaticobiliary ductal dilation |journal=Dig. Dis. Sci. |volume=47 |issue=5 |pages=1091–5 |year=2002 |pmid=12018905 |doi=10.1023/A:1015046309132] [cite journal |author=Christopher V, Thompson M, Hughes S |title=Eosinophilic gastroenteritis mimicking pancreatic cancer |journal=Postgraduate medical journal |volume=78 |issue=922 |pages=498–9 |year=2002 |pmid=12185230 |doi=10.1136/pmj.78.922.498] . Thestomach is the organ most commonly affected, followed by thesmall intestine and the colon [cite journal |author=Naylor A |title=Eosinophilic gastroenteritis |journal=Scottish medical journal |volume=35 |issue=6 |pages=163–5 |year=1990 |pmid=2077646] [cite journal |author=Jimenez-Saenz M, Villar-Rodriguez J, Torres Y, Carmona I, Salas-Herrero E, Gonzalez-Vilches J, Herrerias-Gutierrez J |title=Biliary tract disease: a rare manifestation of eosinophilic gastroenteritis |journal=Dig. Dis. Sci. |volume=48 |issue=3 |pages=624–7 |year=2003 |pmid=12757181 |doi=10.1023/A:1022521707420] .Pathophysiology
Peripheral blood
eosinophilia and elevatedserum IgE are usual but not universal. The damage to thegastrointestinal tract wall is caused by eosinophilic infiltration anddegranulation [cite journal |author=Tan A, Kruimel J, Naber T |title=Eosinophilic gastroenteritis treated with non-enteric-coated budesonide tablets |journal=European journal of gastroenterology & hepatology |volume=13 |issue=4 |pages=425–7 |year=2001 |pmid=11338074 |doi=10.1097/00042737-200104000-00021] .As a part of host defense mechanism,
eosinophil is normally present in gastrointestinalmucosa , though finding in deeper tissue is almost always pathologic [cite journal |author=Blackshaw A, Levison D |title=Eosinophilic infiltrates of the gastrointestinal tract |journal=J. Clin. Pathol. |volume=39 |issue=1 |pages=1–7 |year=1986 |pmid=2869055 |doi=10.1136/jcp.39.1.1] . 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. Foodallergy and variableIgE 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 likeeczema ,asthma etc.Eosinophil recruitment into inflammatory tissue is a complex process, regulated by a number of
inflammatory cytokines . In EGcytokines 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 [cite journal |author=Desreumaux P, Bloget F, Seguy D, Capron M, Cortot A, Colombel J, Janin A |title=Interleukin 3, granulocyte-macrophage colony-stimulating factor, and interleukin 5 in eosinophilic gastroenteritis |journal=Gastroenterology |volume=110 |issue=3 |pages=768–74 |year=1996 |pmid=8608886 |doi=10.1053/gast.1996.v110.pm8608886] . In additioneotaxin has been shown to have an integral role in regulating thehoming of eosinophils into thelamina propria of stomach and small intestine [cite journal |author=Mishra A, Hogan S, Brandt E, Rothenberg M |title=An etiological role for aeroallergens and eosinophils in experimental esophagitis |journal=J. Clin. Invest. |volume=107 |issue=1 |pages=83–90 |year=2001 |pmid=11134183 |doi=10.1172/JCI10224] . In the allergic subtype of disease, it is thought that foodallergens cross the intestinal mucosa and trigger an inflammatory response that includesmast cell degranulation and recruitment of eosinophils [cite journal |author=Pérez-Millán A, Martín-Lorente J, López-Morante A, Yuguero L, Sáez-Royuela F |title=Subserosal eosinophilic gastroenteritis treated efficaciously with sodium cromoglycate |journal=Dig. Dis. Sci. |volume=42 |issue=2 |pages=342–4 |year=1997 |pmid=9052516 |doi=10.1023/A:1018818003002] .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 [cite journal |author=Christopher V, Thompson M, Hughes S |title=Eosinophilic gastroenteritis mimicking pancreatic cancer |journal=Postgraduate medical journal |volume=78 |issue=922 |pages=498–9 |year=2002 |pmid=12185230 |doi=10.1136/pmj.78.922.498] ; 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 [cite journal |author=Shweiki E, West J, Klena J, Kelley S, Colley A, Bross R, Tyler W |title=Eosinophilic gastroenteritis presenting as an obstructing cecal mass--a case report and review of the literature |journal=Am. J. Gastroenterol. |volume=94 |issue=12 |pages=3644–5 |year=1999 |pmid=10606337] [cite journal |author=Tran D, Salloum L, Tshibaka C, Moser R |title=Eosinophilic gastroenteritis mimicking acute appendicitis |journal=The American surgeon |volume=66 |issue=10 |pages=990–2 |year=2000 |pmid=11261632] .
*Mucosal EG (25-100%)is the commonest variety,cite journal |author=Baig M, Qadir A, Rasheed J |title=A review of eosinophilic gastroenteritis |journal=Journal of the National Medical Association |volume=98 |issue=10 |pages=1616–9 |year=2006 |pmid=17052051] [cite journal |author=Lee C, Changchien C, Chen P, Lin D, Sheen I, Wang C, Tai D, Sheen-Chen S, Chen W, Wu C |title=Eosinophilic gastroenteritis: 10 years experience |journal=Am. J. Gastroenterol. |volume=88 |issue=1 |pages=70–4 |year=1993 |pmid=8420276] which presents with features ofmalabsorption andprotein losing enteropathy . Failure to thrive andanaemia may also be present.Lower gastrointestinal bleeding may imply colonic involvement.
*Muscular EG (13-70%) present with obstruction ofgastric outlet or small intestine; sometimes as an obstructing caecal mass orintussusception .
*Subserosal EG (4.5 % to 9 % in Japan and 13 % in the USA) [cite journal |author=Miyamoto T, Shibata T, Matsuura S, Kagesawa M, Ishizawa Y, Tamiya K |title=Eosinophilic gastroenteritis with ileus and ascites |journal=Intern. Med. |volume=35 |issue=10 |pages=779–82 |year=1996 |pmid=8933185 |doi=10.2169/internalmedicine.35.779)] presents with ascites which is usually exudative in nature, abundant peripheral eosinophilia, and has favourable responses tocorticosteroids .
*Other documented features areCholangitis ,pancreatitis [cite journal |author=Lyngbaek S, Adamsen S, Aru A, Bergenfeldt M |title=Recurrent acute pancreatitis due to eosinophilic gastroenteritis. Case report and literature review |journal=JOP |volume=7 |issue=2 |pages=211–7 |year=2006 |pmid=16525206] , eosinophilicsplenitis , acuteappendicitis and giant refractoryduodenal ulcer .Diagnosis
Talley et al.cite journal |author=Talley N, Shorter R, Phillips S, Zinsmeister A |title=Eosinophilic gastroenteritis: a clinicopathological study of patients with disease of the mucosa, muscle layer, and subserosal tissues |journal=Gut |volume=31 |issue=1 |pages=54–8 |year=1990 |pmid=2318432 |doi=10.1136/gut.31.1.54] 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 inascitic 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, buthypoalbuminaemia 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 likeCrohn's disease andlymphoma .The
endoscopic appearance in eosinophilic gastroenteritis is nonspecific; it includes erythematous, friable, nodular, and occasional ulcerative changescite journal |author=Chen M, Chu C, Lin S, Shih S, Wang T |title=Eosinophilic gastroenteritis: clinical experience with 15 patients |journal=World J. Gastroenterol. |volume=9 |issue=12 |pages=2813–6 |year=2003 |pmid=14669340] . Sometimes diffuse inflammation results in complete loss ofvilli , involvement of multiple layers,submucosal oedema andfibrosis . [cite journal |author=Johnstone J, Morson B |title=Eosinophilic gastroenteritis |journal=Histopathology |volume=2 |issue=5 |pages=335–48 |year=1978 |pmid=363591 |doi=10.1111/j.1365-2559.1978.tb01726.x] [cite journal |author=Katz A, Goldman H, Grand R |title=Gastric mucosal biopsy in eosinophilic (allergic) gastroenteritis |journal=Gastroenterology |volume=73 |issue=4 Pt 1 |pages=705–9 |year=1977 |pmid=892374]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
Tc-99m HMPAO-labeledleukocyte 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 [cite journal |author=Lee K, Hahm K, Kim Y, Kim J, Cho S, Jie H, Park C, Yim H |title=The usefulness of Tc-99m HMPAO labeled WBC SPECT in eosinophilic gastroenteritis |journal=Clinical nuclear medicine |volume=22 |issue=8 |pages=536–41 |year=1997 |pmid=9262899 |doi=10.1097/00003072-199708000-00005] [cite journal |author=Imai E, Kaminaga T, Kawasugi K, Yokokawa T, Furui S |title=The usefulness of 99mTc-hexamethylpropyleneamineoxime white blood cell scintigraphy in a patient with eosinophilic gastroenteritis |journal=Annals of nuclear medicine |volume=17 |issue=7 |pages=601–3 |year=2003 |pmid=14651361] .When eosinophilic gastroenteritis is observed in association with eosinophilic infiltration of other organ systems, the diagnosis of
idiopathic hypereosinophilic syndrome should be considered [cite journal |author=Matsushita M, Hajiro K, Morita Y, Takakuwa H, Suzaki T |title=Eosinophilic gastroenteritis involving the entire digestive tract |journal=Am. J. Gastroenterol. |volume=90 |issue=10 |pages=1868–70 |year=1995 |pmid=7572911] .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 (anantihistamine ), andmontelukast (a selective, competitive leukotriene receptor antagonist) have been proposed, centering around an allergic hypothesis, with mixed results [cite journal |author=Barbie D, Mangi A, Lauwers G |title=Eosinophilic gastroenteritis associated with systemic lupus erythematosus |journal=J. Clin. Gastroenterol. |volume=38 |issue=10 |pages=883–6 |year=2004 |pmid=15492606 |doi=10.1097/00004836-200411000-00010] . An elimination diet may be successful if a limited number of food allergies are identified [cite journal |author=Katz A, Twarog F, Zeiger R, Falchuk Z |title=Milk-sensitive and eosinophilic gastroenteropathy: similar clinical features with contrasting mechanisms and clinical course |journal=J. Allergy Clin. Immunol. |volume=74 |issue=1 |pages=72–8 |year=1984 |pmid=6547462 |doi=10.1016/0091-6749(84)90090-3] .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 [cite book | last = Guandalini | first = Stefano | title = Essential Pediatric Gastroenterology and Nutrition | publisher = McGraw-Hill Professional | location = City | year = 2004 | isbn = 0071416307 Page 210.] . 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
*Infantileformula protein intolerance See also
Allergy Gastroenteritis Malabsorption References
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