Eosinophilic Granulomatosis With Polyangiitis With Diffuse Gastrointestinal Involvement

Abstract

Introduction: Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare systemic, small, and medium-sized-vessel vasculitis. Gastrointestinal (GI) symptoms like diarrhea and abdominal pain are common; however, there are few reports of histological evidence of GI involvement. We report a case of a patient on treatment for EGPA who presented with recurrent small bowel obstruction and choledocholithiasis. A 64-year-old gentleman with a 12-year history of EGPA presented to our gastroenterology clinic with recurrent choledocholithiasis and 3 episodes of partial small bowel obstruction in a period of 6 weeks. The vasculitis was stable on methotrexate and prednisone, with serial stable inflammatory markers and absolute eosinophils prompting a reduction in immune suppression 3 months prior to presentation. On presentation, new lower extremity purpuric lesions were detected. Elevated inflammatory markers and eosinophilia were detected. MR enterography showed mechanical obstruction proximal to the ileocecal valve (Figure 1). ERCP and extended upper enteroscopy showed a dilated biliary tree, nodular mucosa in the gastric antrum, and duodenal polyps. A retrograde balloon-assisted enteroscopy showed thickened mucosal folds in the distal ileum corresponding to the abnormal MRI. Biopsies showed inflammatory expansion of the lamina propria, including the presence of increased eosinophils. Medical management with increased dose of MTX/prednisone was elected. After 2 months of being totally asymptomatic on higher dose immune suppression, the patient passed away. This was attributed to known pre-existing heart disease likely due to EGPA involvement. Although GI involvement of EGPA is relatively common, not many cases have reported the specific anatomical involvement through histopathological evaluation. The literature has reported cases of colitis, and gastric and colonic ulcers. We report a case of small bowel obstruction with histopathologic findings that also had widespread GI involvement. Awareness of the clinical presentation of GI involvement may allow more timely and effective management.Figure 1