Zebras Do Exist: Churg-Strauss with GI Involvement

Abstract

Purpose: A 57-year-old woman with recent diagnosis of asthma was admitted with progressive, diffuse abdominal pain for several weeks. The patient also reported non-bloody diarrhea, nausea without emesis, and early satiety. She denied fevers, recent travel, and family history of inflammatory bowel disease (IBD). On physical examination, vital signs were normal and abdominal exam was notable for mild diffuse tenderness to palpation. Admission labs revealed a white blood cell count of 20K with 56% eosinophils. Given diarrhea, abdominal pain, and eosinophilia, diagnosis of IBD was felt to be most likely with other considerations being parasitic infection, allergic reaction, connective tissue disease, neoplasm, and hypereosinophilic syndrome. Stool studies were negative for parasites. ESR and CRP were significantly elevated. CT abdomen showed wall thickening of the distal duodenum and jejunum again suggesting possible small bowel IBD. EGD was performed and was endoscopically normal to the duodenum with random biopsies showing no significant pathology. While admitted, the patient developed dyspnea with intermittent hypoxia; chest x-ray was performed and showed new pulmonary infiltrates. The patient continued to have persistent abdominal symptoms therefore endoscopy was repeated and this time revealed scattered duodenal and jejunal erosions/small ulcers and gastric erythema. Biopsies obtained from the stomach revealed eosinophilic vasculitis with thrombosis involving the small lamina propria blood vessels. The diagnosis of Churg-Strauss was confirmed with additional testing and the patient was treated with high-dose corticosteroids with resolution of all abdominal symptoms. This case illustrates several learning points including the importance of evaluating a broad differential diagnosis. IBD is a much more common cause of abdominal pain and diarrhea than gastrointestinal vasculitis, however, based on the clinical setting, even “zebras” must be considered. Our patient had a diagnosis of asthma in lateadulthood, significant peripheral eosinophilia, varying pulmonary complaints, and transient pulmonary infiltrates, all of which are consistent with Churg-Strauss. The case also emphasizes the need for repeat endoscopy with biopsy in cases that remain enigmatic as initial biopsies can be non-diagnostic due to sampling variation or inadequate depth. In our case, only after repeat biopsies could a final diagnosis be made and appropriate treatment initiated.