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Case Reports
. 2014 Oct 28;8(3):329-36.
doi: 10.1159/000369129. eCollection 2014 Sep.

Eosinophilic granulomatosis with polyangiitis and diffuse gastrointestinal involvement

Diana L Franco  1 , Kevin Ruff  2 , Lester Mertz  3 , Dora M Lam-Himlin  1 , Russell Heigh  2
Affiliations
Case Reports

Eosinophilic granulomatosis with polyangiitis and diffuse gastrointestinal involvement

Diana L Franco et al. Case Rep Gastroenterol. .

Abstract

Eosinophilic granulomatosis with polyangiitis (EGPA), formerly named Churg-Strauss syndrome, is a rare systemic small- and medium-sized-vessel vasculitis, characterized by the presence of severe asthma as well as blood and tissue eosinophilia. Gastrointestinal (GI) symptoms, like diarrhea and abdominal pain, are common; however, there are few reports of histologic evidence of GI involvement. We report the case of a patient on treatment for EGPA who presented with recurrent small bowel obstruction and choledocholithiasis. Biopsies of the esophagus, small bowel and common bile duct showed diffuse eosinophilia, with clear EGPA in the GI tract. Improved awareness of GI EGPA may allow for timely management of this disorder.

Keywords: Cholangitis; Eosinophilic granulomatosis with polyangiitis; Gastrointestinal tract; Small bowel obstruction.

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Figures

Fig. 1
Fig. 1
Magnetic resonance enterography. The arrow points to the distal small bowel thickening due to mechanical obstruction proximal to the ileocecal valve.
Fig. 2
Fig. 2
a 10× magnification. Hematoxylin & eosin stain of the common bile duct shows marked expansion of the lamina propria with chronic inflammation (original magnification 100×). b 60 times higher magnification shows that the inflammatory infiltrate is composed of mixed chronic inflammatory cells including plasma cells, lymphocytes, and a striking increase in bright pink eosinophils (original magnification 600×).
Fig. 3
Fig. 3
a 20× magnification. Hematoxylin & eosin stain of the ileum shows features of chronic damage including mild villous blunting and mucinous metaplasia of the surface epithelium (bracket). Note also the mild expansion of the lamina propria by inflammatory cells (original magnification 200×). b 60× magnification. While scattered, single eosinophils are a normal finding in the crypt epithelium, this example shows an increase of intraepithelial eosinophils with multiple eosinophils crossing the basement membrane, some of which are back-to-back (arrows) (original magnification 600×).
See this image and copyright information in PMC

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