Eosinophilic gastroenteritis: Approach to diagnosis and management

Table 1 Eosinophilic gastroenteritis severity upon presentation

Initial findings	Mild	Moderate	Severe	Complicated
Clinical
Abdominal pain	Mild	Moderate	Severe
Vomiting	Mild (< 3/d)	Moderate (3-7/d)	protracted (> 8/d)
Diarrhea	< 6 BM/d	6-12 BM/d	> 12 BM/d
Weight loss1[35]	Non-significant	1 wk 1%-2% 1 mo 5% 3 mo 7.5% 6 mo 10%	1 wk > 2% 1 mo > 5% 3 mo > 7.5% 6 mo > 10%
Laboratory
Alb, g/dL	> 3	2.5-3	< 2.5
HB, g/dL[36]	9.5-11	8-9.5	< 8
AEC, cells/μL[37]	< 1500	1500-5000	> 5000
Radiologic
Ascites	None or mild	Moderate volume	Large volume	Perforation
Intestinal wall thickening[38]	Mild (1-2 cm) Focal (< 10 cm)	Marked (> 2 cm), segmental (10-30 cm)	Sub-occlusion, extensive (> 30 cm)	Occlusion Intussusception
Endoscopy
Mucosal inflammation[39]	Normal or mild erythema	Moderate	Severe with pseudo-polyps/bleeding	GOO Pyloric stenosis
Histology
Structural damage2[34]	Minimal	Moderate	Severe

¹Percent weight change = [(usual weight - actual weight)/(usual weight)] × 100

²Subjective assessment by expert pathologist. AEC: Absolute eosinophilic count; Alb: Albumin; GOO: Gastric outlet obstruction; HB: Hemoglobin.

Table 2 Published cases of eosinophilic gastroenteritis treated with budesonide

Ref.	Patient no.	Intestinal layer	Location	Previous treatment	Response
Russel et al[52], 1994	1	Mucosal	Ileum and cecum	Intolerant to steroids Failure of cromolyn sodium and mesalazine	Efficacy comparable to steroids over 5 mo
Tan et al[53], 2001	1	Full thickness with ascites	Antrum	Steroid dependent	Remission (+) over 2 yr
Siewert et al[54], 2006	1	Mucosal	Duodenum to ileum	None	Response (+)
Lombardi et al[55], 2007	1	Mucosal + submucosal	Ileum	Relapse after stopping budesonide and cromolyn sodium	Remission (+) on budesonide alone over 4 mo
Elsing et al[56], 2007	1	Muscular	Jejunum	Surgery + steroids for relapse	Remission (+) over 3 mo
Shahzad et al[57], 2011	1	Mucosal	Antrum + colon	None	Response (+)
Busoni et al[58], 2011	5	Mucosal	Lower + upper GI tract	Prednisone/methylprednisolone	Remission (+)
Lombardi et al[59], 2011	1	Muscular	Pyloric stenosis	Methylprednisolone	Remission (+) over 6 mo
Müller et al[26], 2014	1	Mucosal	Duodenum + colon + ileum	None	50% response (combined with 6-food elimination diet)
Wong et al[15], 2015	1	Mucosal +/- serosal or muscular	-	None	Recurrent symptoms

GI: Gastrointestinal.

Table 3 Published cases of eosinophilic gastroenteritis treated with montelukast

Ref.	Patient no.	Intestinal layer	Location	Previous treatment	Response
Neustrom et al[63], 1999	1	Mucosal	Esophagus + stomach + small intestine	Failure of response to elimination diet, cromolyn sodium, ranitidine and hydroxyzine	Clinical and histologic response (+)
Schwartz et al[64], 2001	1	Serosal	Duodenum	Steroid dependent	Remission (+) over 4 wk
Lu et al[65], 2003	2	Mucosal	-	Steroid dependent	1 → Not effective
					2 → Partial response with tapering of prednisone to 10 mg/d
Vanderhoof et al[66], 2003	8	Mucosal	Esophagus (n = 4) Duodenum (n = 2) Colon (n = 2)	Failure of standard therapies	Clinical response (+) within 1 mo
Copeland et al[9], 2004	1	Mucosal	Stomach	Steroid refractory EGE (also receiving 6MP and 5ASA for UC)	Not effective
Friesen et al[67], 2004	40	Mucosal	Duodenum	None	Response (+) within 2 wk
Quack et al[68], 2005	1	Serosal	Ileum	Steroid dependent	Remission (+) over 2 yr
Urek et al[21], 2006	1	Serosal	Ileum	Steroid dependent	Response (+) within 4 wk
De Maeyer et al[69], 2011	1	-	-	Steroid dependent	Response (+)
Tien et al[5], 2011	12	Mucosal	Stomach + duodenum + colon + esophagus	4 → None 8 → Steroid dependent	Remission (+) over 12 mo 4/8 → Successful steroid tapering 3/8 → Not effective 1/8 → Lost to follow-up
Selva Kumar et al[70], 2011	1	Mucosal	Small intestine	Unresponsive to standard therapy	Response (+)
Müller et al[26], 2014	2	Mucosal (+/- serosal or muscular)	Stomach + small intestine	1 and 2 → Steroid dependent	1 → Remission (+) in combination with low-dose prednisone 2 → Remission (+) (off steroids)
Wong et al[15], 2015	2	Mucosal (+/- serosal or muscular)	-	1: Steroid dependent 2: None	Remission (+) for 36 mo (in combination with prednisone) Asymptomatic for 10 mo

5ASA: 5-Aminosalicylic acid; 6MP: 6 Mercaptopurine; UC: Ulcerative colitis.

Table 4 Published cases of eosinophilic gastroenteritis treated with cromolyn sodium

Ref.	Patient no.	Intestinal layer	Location	Previous treatment	Response
Moots et al[71], 1988	1	Mucosal +/- muscular	Small intestine + colon	Prednisone, cyclophosphamide	Response (+) in 10 wk Maintenance over 2.5 yr
Talley et al[4], 1990	3	Mucosal	-	None	1 → Response (+) 2 → No response
Di Gioacchino et al[72], 1990	2	Mucosal	Stomach + duodenum	None	Clinical and histologic response (+) after 4-5 mo
Beishuizen et al[73], 1993	2	Mucosal	Upper gastrointestinal tract	Steroids	Prolonged response (+)
Van Dellen et al[74], 1994	1	Mucosal	Stomach + duodenum	Elemental diet (poorly tolerated)	Response (+)
Russel et al[52], 1994	1	Mucosal	Ileum + colon	Steroid dependent	None (failure to taper steroids)
Pérez-Millán et al[75], 1997	1	Serosal	Duodenum	None	Response (+) over 6 mo
Suzuki et al[76], 2003	1	Mucosal	Stomach + duodenum	Targeted elimination diet (poorly tolerated)	Response (+) (in combination with ketotifene)
Sheikh et al[77], 2009	3	Mucosal Mucosal Mucosal +/- muscular	Esophagus + stomach + duodenum Stomach + duodenum + colon Esophagus + stomach + duodenum + colon	Steroid refractory None Steroid dependent	Not effective Partial response Response (+) with tapering of prednisone over 6 mo