Clinical Challenges

Clinical Challenges - June 2017
What's Your Diagnosis?

What’s your diagnosis?

By Ravi B. Parikh, MD, George A. Alba, MD, and Lawrence R. Zukerberg, MD. Published previously in Gastroenterology (2013;144;272, 467).

A 36-year-old woman, originally from Haiti, presented to the emergency department with 2 weeks of abdominal distention, diarrhea, and blood-tinged emesis. She had given birth to her first child by uncomplicated cesarean section 9.5 weeks earlier. There was no history of recent travel, diet change, or sick contacts. She denied alcohol, tobacco, or illicit drug use and was not taking any medications or supplements. She was allergic to chloroquine (itchiness) and had no history of atopy. She was not aware of any family history of liver disease or allergy, although her paternal history was unknown.

Upon admittance to the general medicine service, the patient was afebrile and hemodynamically stable. She did not have any stigmata of chronic liver disease. Her abdomen was distended and diffusely tender with rebound tenderness and guarding (Figure A). Serum studies were notable for white blood cell count of 14.5 x 103/microL, with 46% eosinophils (absolute count 6660/mm3). Other values, including serum human chorionic gonadotropin, were normal.

Computed tomography of the abdomen and pelvis (Figure B) showed a large amount of abdominal and pelvic ascites (arrow) with mild small bowel wall thickening. There was no evidence of organomegaly or vessel thrombosis. Subsequent diagnostic paracentesis demonstrated an exudative effusion with total nucleated cells 4,545/mL, with 82% eosinophils. Large-volume paracentesis of 4,000 mL of straw-colored fluid relieved the patient’s abdominal pain. Fluid bacterial and tuberculosis cultures were negative, and cytology showed no evidence of malignancy. Peripheral blood smear was unremarkable. Stool culture, stool ova and parasites, urine culture, and blood culture were all negative.
Because of these findings, the gastroenterology service was consulted. Esophagogastroduodenoscopy and colonoscopy showed mild rectal mucosal erythema (arrow) without masses, bleeding, ulcers, or polyps (Figure C).

What is the diagnosis? What is the appropriate management?


 

Answer: Eosinophilic gastroenteritis

Colonic (Figure D) and esophageal (Figures E and F) mucosal biopsies were obtained, which showed dense eosinophilic infiltrate of the esophageal and rectal submucosa and the rectal deep mucosa. These findings were consistent with eosinophilic gastroenteritis (EGE), mural type. She was empirically treated with 2 doses of ivermectin given the concern for possible underlying parasitic infection given her country of origin, and she was started on oral prednisone 40 mg/d. Eosinophilia and symptoms improved rapidly with this regimen. One month after discharge, her parasitic serology was notable for antifilarial immunoglobulin (Ig) G and IgG4 being positive. At 2-month follow-up, she felt well and denied any abdominal pain or distention with resolution of her peripheral eosinophilia.
The diagnosis of EGE is usually made by endoscopic biopsy showing proliferation of eosinophils in areas of the gastrointestinal tract where eosinophils are uncommon (e.g., esophagus, small bowel).1 It is associated with allergy or atopy, and eosinophil-predominate ascites is a rare presentation of EGE.2 Eosinophilic ascites in the context of postpartum EGE has been described at least twice in case reports.3 It should be noted that eosinophilic infiltration of the gastrointestinal tract may be present in certain conditions, including IgE-mediated food allergies and inflammatory bowel disease. Although certain dietary restrictions can rarely lead to resolution of EGE, systemic steroids are most often used and lead to improved symptomatic response.

Our patient’s positive filarial serology, although not associated with EGE in the literature, is the first known documented association between likely filariasis and EGE. She is presently being further evaluated for active filarial parasitemia and consideration of diethylcarbamazine therapy.

Acknowledgments

The authors thank Dr. Jay Luther for his guidance and manuscript review and Dr. Daniel Pratt for obtaining images.

References

1. Chen, M.J., Chu, C.H., Lin, S.C., et al. Eosinophilic gastroenteritis: clinical experience with 15 patients. World J Gastroenterol. 2003;9:2813-6.

2. Hepburn, I.S., Sridhar, S., Schade, R.R. Eosinophilic ascites, an unusual presentation of eosinophilic gastroenteritis: a case report and review. World J Gastrointest Pathophysiol. 2010;1:166-70.

3. Ogasa, M., Nakamura, Y., Sanai, H., et al. A case of pregnancy associated hypereosinophilia with hyperpermeability symptoms. Gynecol Obstet Invest. 2006;62:14-6.

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